Publications

A practical approach to preventing surgical cancellation in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: tonsillectomy between febrile episodes: our experience.

Kobayashi M, Mochizuki I, Nemoto C , et al. · Journal of anesthesia · 2026

Perinatal and early childhood determinants of disease persistence in PFAPA.

Benderlioğlu E, Efeoğlu Gülsoy G, Özçelik E , et al. · Pediatric research · 2026

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) is a common childhood autoinflammatory disease. This study investigated the influence of early-life factors on attack fr...

Before calling it FMF: MEFV variants of uncertain significance in autoinflammatory disease.

Cam V, Cingoz E, Ercan Emreol H , et al. · Rheumatology (Oxford, England) · 2026

Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by recurrent febrile attacks and serositis, with a high prevalence and carrier frequency of MEFV variants in Eastern Med...

Differentiating Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) and Syndrome of Undifferentiated Recurrent Fever (SURF): A Comparative Study of Clinical Features, Treatment Response, and Remission.

Vyzhga Y, Goh IY, Garibeh E , et al. · The Journal of rheumatology · 2026

To apply proposed classification criteria for periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) and syndrome of undifferentiated recurrent fever (SURF) to a heterogeneous coh...

Decision Tree Analysis as a Preliminary Evidence-Based Tool for Identifying the Syndrome of Undifferentiated Recurrent Fever in Children Compared With Hereditary Recurrent Fevers and Periodic Fever, Aphthosis, Pharyngitis, and Adenitis Syndrome.

Papa R, Bovis F, Federici S , et al. · Arthritis & rheumatology (Hoboken, N.J.) · 2026

To develop evidence-based criteria to classify patients with syndrome of undifferentiated recurrent fevers (SURF). One hundred twelve patients with SURF observed in a single tertiary referral center w...

Parent Perspectives on the Diagnosis, Management, and Family Impact of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome.

Londono L, Bell KJ, Stagliano DR , et al. · Military medicine · 2026

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) is a rare, self-resolving, under-recognized autoinflammatory condition impacting young children. There is limited manage...

Cluster Analysis in the Differential Diagnosis of PFAPA Syndrome.

Karali Y, Karali Z, Ercan I , et al. · Clinical pediatrics · 2026

The differential diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome from recurrent upper respiratory tract infections (URTIs) and familial Mediterran...

How I Treat: Haploinsufficiency of A20.

Li J, Zhou JY, Carpio Tumba M , et al. · Journal of human immunity · 2026

Haploinsufficiency of A20 (HA20) is a primary immune regulation disease caused by heterozygous loss-of-function variants in , resulting in unchecked inflammatory signaling. HA20 is a highly heterogene...

Cimetidine for Pediatric Use in Japan: Rising Prescriptions for a New Indication.

Oshima K, Inage E, Toriumi S , et al. · The Tohoku journal of experimental medicine · 2026

Cimetidine is effective for treating periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, primarily in children, and is covered by public insurance in Japan. Howev...

Accurate Diagnosis of Familial Mediterranean Fever Improved Quality of Life for a Patient and Her Family: A Case Report.

Kurihara M, Igarashi T, Ishii R , et al. · Journal of Nippon Medical School = Nippon Ika Daigaku zasshi · 2026

Periodic fever syndrome is diagnosed on the basis of duration of fever, associated symptoms, and blood and genetic test results. During a 6-month period, a 3-year-old girl experienced monthly fever (3...

Description of a Large Family with Periodic Fever Carrying a Variant in Gene: A Possible Novel Modulator of Inflammation in Autoinflammatory Diseases.

Buttarelli M, Rapari G, Riccio M , et al. · International journal of molecular sciences · 2026

Autoinflammatory diseases involve recurrent systemic inflammation caused by dysregulated innate immunity, arising from genetic or multifactorial mechanisms, as seen in periodic fever, aphthous stomati...

Colchicine prophylaxis in pediatric PFAPA: a systematic review.

Dipasquale RF, Sinopoli P, Mendicino A , et al. · European journal of pediatrics · 2026

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever syndrome in children. On-demand corticosteroids stop attacks but may shorten fever-fre...

Serum amyloid A as predictive factor in PFAPA syndrome attack.

Kazanasmaz H, Gündoğmuş F, Eroğlu EC , et al. · Medicina clinica · 2026

This study aimed to assess the diagnostic value of serum amyloid A (SAA) levels during attacks of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. A retrospective analy...

Evaluation of clinical responses to probiotics and colchicine in PFAPA patients based on autoinflammatory diseases activity index (AIDAI) scores.

Yabanci Erten ES, Koru L, Kaya F , et al. · Pediatric research · 2026

Data on prophylaxis in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) are limited for probiotics. We aimed to evaluate clinical responses of probiotic an...

Phenotypes of patients with more than one autoinflammatory disease-associated gene variant: overlapping and mixed autoinflammatory disorders.

Amikishiyev S, Kalaycı T, Deniz R , et al. · Rheumatology (Oxford, England) · 2026

Genetic screening for systemic autoinflammatory disorders (SAIDs) often does not yield a definite diagnosis based on pathogenic variants. Instead, many patients are found to carry combinations of vari...

Validity of the Eurofever/PRINTO genetic FMF classification criteria in FMF and PFAPA patients carrying non-confirmatory MEFV gene mutations.

Ilgaz Tüzen H, Yardımcı Y, Güneş Yılmaz S , et al. · Clinical rheumatology · 2026

Familial Mediterranean Fever (FMF) and Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome are autoinflammatory diseases that may present with similar clinical symptoms. Th...

Casting light on the potential connection: exploring the relationship between periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and Behҫet in the Druze population in Israel.

Gharra A, Amarilyo G, Harel L , et al. · Orphanet journal of rare diseases · 2026

To characterize the clinical presentation of PFAPA and the response to treatment in an ethnic subgroup- Druze and analyze the differences compared to other ethnic groups. Retrospective data were colle...

CARD8 variant-mediated inflammasome activation in a case of refractory Behçet disease: from PFAPA to atypical neurovascular involvement.

Pai YH, Lin KH, Yang SC , et al. · Rheumatology (Oxford, England) · 2026

Colchicine prophylaxis efficacy in PFAPA syndrome: A comparative cohort study.

Kasem AliSliman R, Regev S, Ghanim B , et al. · Seminars in arthritis and rheumatism · 2026

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome is the most common periodic fever disorder in children, causing recurrent debilitating episodes that impose a substantial bu...

The etiological spectrum of pediatric FUO and clinical management of PFAPA/SURF: a ten-year retrospective study.

Guan C, Zhou Q, Sun J , et al. · Frontiers in immunology · 2026

This study aimed to define the etiologies and clinical features of pediatric Fever of unknown origin (FUO), focusing on periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA)...

Clinical, immunological characterisation and treatment response of patients with syndrome of undifferentiated recurrent fever in Chinese children and adolescents: a single-centre cohort study.

Chen X, Xing W, Li Y , et al. · Frontiers in immunology · 2026

Syndrome of undifferentiated recurrent fevers (SURF) is a heterogeneous disorder characterised by recurrent fevers and autoinflammation in the absence of a confirmed molecular diagnosis of hereditary...

Cytokine profiles and laboratory parameters as indicators to distinguish children with PFAPA and bacterial infection.

Zhu X, Yang Z, Huang Y , et al. · Frontiers in immunology · 2026

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is characterized by recurrent febrile episodes associated with one or more of the symptoms described by the acr...

Understanding PFAPA Syndrome in Palestine: A Retrospective Cohort Analysis of Epidemiological and Clinical Data.

Abunejma FM, Wazwaz R, Qawasma R , et al. · Mediterranean journal of rheumatology · 2025

Despite Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy being widely acknowledged as a clinical entity and considered one of the most prevalent autoinflammatory diseases, there remain...

Diagnostic role of Galectin-3 with routine blood parameters in PFAPA syndrome: prospective cohort study.

Dagli SB, Gumussoy M, Kanik A , et al. · International journal of pediatric otorhinolaryngology · 2025

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome in Children-From Pathogenesis to Treatment Strategies: A Comprehensive Review.

Anselmi F, Dusser P, Kone-Paut I · Paediatric drugs · 2025

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent periodic fever syndrome in non-Mediterranean children, usually manifesting before the age...

Exploring the significance of vitamin D insufficiency in the periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: a single-center retrospective assessment during the decade 2014-2024.

Rigante D, Manna R, Candelli M · Internal and emergency medicine · 2025

The underlying mechanisms responsible for the periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome are unknown. The main purpose of this study was to retrospectivel...

EULAR/ACR classification criteria for paediatric chronic nonbacterial osteomyelitis (CNO).

Zhao Y, Oliver MS, Schnabel A , et al. · Annals of the rheumatic diseases · 2025

To develop and validate classification criteria for paediatric chronic nonbacterial osteomyelitis (CNO) jointly supported by the European Alliance of Associations for Rheumatology (EULAR) and the Amer...

Clinical and Imaging Characteristics of Posterior Scleritis in Children.

Katzir A, Amer R · Ocular immunology and inflammation · 2025

Posterior scleritis (PS) is a rare phenotype of scleritis. Comprehensive epidemiological studies on PS in children are limited. We aimed to report on its clinical and imaging features in one of the la...

PFAPA meets Behçets: a rare overlap in a paediatric patient.

Gupta SK, Rana S, Swami K , et al. · Clinical and experimental dermatology · 2025

Vaccination coverage, immune responses, and clinical characteristics in children with PFAPA syndrome: a monocentric prospective study.

Sallansonnet E, Gualtieri R, Margot H , et al. · Pediatric rheumatology online journal · 2025

PFAPA syndrome (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) is the most common periodic fever condition in children. Vaccination in this population can be challenging due to concer...

Autoinflammatory periodic fever syndromes in preschoolers: neurodevelopmental, behavioral, and maternal psychosocial outcomes.

Ömercioğlu E, Özçelik E, Akpınar F , et al. · European journal of pediatrics · 2025

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF) are the most common pediatric autoinflammatory syndromes, yet their deve...

Syndrome of undifferentiated recurrent fever (SURF): a multicenter real-world experience from Türkiye.

Güngörer V, Ünal D, Çakan M , et al. · Clinical rheumatology · 2025

Syndrome of undifferentiated recurrent fever (SURF) is an autoinflammatory disorder that is recognised in an increasing number of patients. In this study, we aimed to assess the data of SURF patients...

Corrigendum to "Diagnostic role of Galectin-3 with routine blood parameters in PFAPA syndrome: prospective cohort study" [Int. J. Pediatric Otorhinolaryngol. 196 (2025) 112478].

Dagli S, Gumussoy M, Kanik A , et al. · International journal of pediatric otorhinolaryngology · 2025

Surgical Treatments for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: Systematic Review and Network Meta-analysis.

Noy R, Barzilai R, Cohen JT , et al. · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery · 2025

To compare surgical versus medical treatment approaches for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome. PubMed, Embase, Web of Science, and Cochrane. We followed t...

Colchicine treatment in PFAPA: how long should we wait for the clinical response?

Koru L, Erten ESY, Dursun HK , et al. · European journal of pediatrics · 2025

We aimed to evaluate the efficacy of prophylactic colchicine treatment in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) using the Auto-Inflammatory Diseases Activity...

Clinical, Immunologic, and Genetic Characteristics in Patients With Syndrome of Undifferentiated Recurrent Fevers.

Macaraeg M, Baker E, Handorf E , et al. · Arthritis & rheumatology (Hoboken, N.J.) · 2025

Syndrome of undifferentiated recurrent fevers (SURF) is characterized by recurrent fevers and autoinflammation without a confirmed molecular diagnosis of a hereditary recurrent fever syndrome, and not...

Efficacy of partial tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome: a STROBE retrospective observational study.

Banh Chong T, Sagot O, Alexis M , et al. · European annals of otorhinolaryngology, head and neck diseases · 2025

PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) or Marshall syndrome is the most frequent cause of recurrent auto-inflammatory fever in children. Frequent episodes impair the child'...

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome: An Update.

Michailou M, Perdikogianni C · Children (Basel, Switzerland) · 2025

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an autoinflammatory disorder of unknown genetic etiology typically characterized by recurrent fever episodes, pharyngi...

Case Report: PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis) Syndrome With a Novel TNFAIP3 Mutation.

Deng J, Guo H, Kong R , et al. · Immunity, inflammation and disease · 2025

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome has been considered as a childhood syndrome. Its etiopathogeny is unknown however, currently considered as auto...

Assessment of Attack Frequency in PFAPA Patients Based on Therapeutic Modalities.

Kalayci F, Yigit M, Kuruc AI , et al. · Journal of paediatrics and child health · 2025

This study aims to present the clinical characteristics of patients with PFAPA syndrome, and to compare the effects of corticosteroid usage, colchicine prophylaxis, and tonsillectomy on the frequency...

Long-Term Symptoms in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome after Tonsillectomy.

Moberg T, Rydenman K, Berg S , et al. · The Journal of pediatrics · 2025

To investigate the initial and long-term clinical course of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy regarding fever episodes and no...

Corticosteroid use in PFAPA syndrome: clinical practice data from the JIR-CliPS Survey Study and a comprehensive literature review.

Batu ED, Sener S, Rodrigues M , et al. · Rheumatology (Oxford, England) · 2025

CS are used to abort disease flares in periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. We aimed to obtain a global overview of physicians' CS usage strategies...

Colchicine efficacy on oral ulcers caused by Behçet's spectrum disorders including idiopathic recurrent aphthous stomatitis, PFAPA, and Behçet's Disease.

Aydin Z, Kaya F, Kucuk E , et al. · European journal of pediatrics · 2025

Recurrent aphthous stomatitis (RAS) is a common condition in pediatric age. Since there are significant genetic commonalities among idiopathic cases, periodic fever, aphthous stomatitis, pharyngitis,...

Deep Proteomics Analysis Unravels the Molecular Signatures of Tonsillar B Cells in PFAPA and OSAS in the Pediatric Population.

Kharrat F, Balasan N, Ura B , et al. · International journal of molecular sciences · 2025

Tonsils are secondary lymphoid organs that play a crucial role in the immunological response, with B cells being a major component involved in both innate and adaptive immunity. Periodic fever, aphtho...

Systemic autoinflammatory disease diagnoses in a single year from a UK national referral centre.

Caterson HC, Papadopoulou C, Peet C , et al. · Rheumatology (Oxford, England) · 2025

The diagnosis of systemic autoinflammatory diseases (SAIDs) is frequently missed or delayed. The establishment of specialist clinics and the availability of genetic testing enables increased diagnosis...

Features Associated with Response to Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome in Children.

Manthiram K, Ortega-Villa AM, Lapidus S , et al. · The Journal of pediatrics · 2025

To identify clinical features associated with response to tonsillectomy among children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome and to determine op...

Symptom course of PFAPA syndrome.

Labouret M, Elhani I, Cavelot S , et al. · Pediatric rheumatology online journal · 2025

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of autoinflammatory periodic fever in children. It is generally considered to be a self...

Letter on the article "Efficacy of partial tonsillectomy in periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome: A STROBE retrospective observational study" by Banh Chong T, et al. Eur Ann Otorhinolaryngol Head Neck Dis 2025;142:115-20.

Wang Y, Tang X · European annals of otorhinolaryngology, head and neck diseases · 2025

Late-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome: A Cue for Antimicrobial Stewardship.

Parvathy K, Reghukumar A, Gurudas A , et al. · Cureus · 2025

PFAPA is an auto-inflammatory syndrome, characterised by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis, most commonly described in children. We report two cases of late-onset...

An Italian multidisciplinary Delphi Consensus on new insights about the clinical relevance of Pidotimod.

Marseglia G, Gelardi M, Marchisio P , et al. · Multidisciplinary respiratory medicine · 2025

Pidotimod is a synthetic dipeptide that modulates both innate and acquired immunity, with the highest number of scientific publications (as documented in PubMed) which have demonstrated its efficacy a...

Predictive factors for therapeutic response and cluster analysis in syndrome of undifferentiated recurrent fever (SURF).

Palmeri S, Ponzano M, Recchi G , et al. · RMD open · 2025

Syndrome of undifferentiated recurrent fever (SURF) refers to a group of recurrent fevers without a clear monogenic cause. Clinical spectrum, treatment response predictors and management strategies re...

Comprehensive Perioperative Management of PFAPA Syndrome: Insights From Clinical Cases.

Uslu A, Çekmen N · Journal of perianesthesia nursing : official journal of the American Society of PeriAnesthesia Nurses · 2025

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, a common cause of recurrent fever in childhood, presents a challenge in both diagnosis and management. While i...

The first proteomics analysis of tonsils in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA).

Mutlu F, Kasap M, Yaprak Bayrak B , et al. · Pediatric research · 2025

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a recurrent fever syndrome. The exact etiopathogenesis of PFAPA syndrome remains unknown. Biological fluids or tissue...

Quality of Life and Psychological Functioning in Children with PFAPA Syndrome.

García-Soto XR, Villanueva-Alameda MI, Fernández-Solana J , et al. · Pediatric reports · 2025

This study analyzes the impact of PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) on health-related quality of life (HRQoL) and the psychological functioning of childre...

Case Report: Successful Remission With Upadacitinib in Behçet's Syndrome Following a History of PFAPA Syndrome.

Oka H, Sumitomo S, Yamashita D , et al. · International journal of rheumatic diseases · 2025

Recent studies have revealed a shared genetic background and pathogenesis between Behçet's syndrome (BS) and periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. T...

Neglected adult PFAPA syndrome.

Zhang A, Zhang R, Jiang Y , et al. · Radiology case reports · 2025

Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA) syndrome is rarely seen in adults. The diagnosis is challenging, and the majority of cases are misdiagnosed early. Here w...

Surgical management for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: a systematic review and meta-analysis.

Cavalcante LFF, Costa G, Battistuta SM , et al. · Jornal de pediatria · 2025

This systematic review and meta-analysis aimed to evaluate the efficacy of surgical management, specifically total and partial tonsillectomy, for Periodic Fever, Aphthous Stomatitis, Pharyngitis, and...

A Case of Lipschütz Ulcers in a 13-Year-Old Girl With PFAPA Syndrome.

Parker R, Johns HR, Pourciau C · Pediatric dermatology · 2025

Lipschütz ulcers (LU), also known as reactive non-sexually related acute genital ulcerations, are an uncommon condition that primarily affects sexually inactive girls under the age of 20 years. In thi...

Clinical characteristics and quality of life in children with PFAPA syndrome and Behçet's disease.

Gürel Bedir A, Kilic SS · Rheumatology advances in practice · 2025

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome and Behçet's disease (BD) are non-monogenic autoinflammatory disorders with common clinical characteristics and gen...

Otorhinolaryngological manifestations of autoinflammatory diseases. Systematic review.

Mallo-Miranda MV, Morales-Angulo C · Acta otorrinolaringologica espanola · 2025

To detail the main otorhinolaryngological manifestations of autoinflammatory diseases, aiming to contribute to early diagnosis and treatment. Searches were conducted in PubMed, LILACS, Cochrane Librar...

The Potential Role of Cell-Death Mechanisms in the Pathogenesis of Familial Mediterranean Fever Attacks: Granzyme A and Beyond.

Yaglikara E, Boluk O, Bayindir Y , et al. · Diagnostics (Basel, Switzerland) · 2024

FMF is the most common autoinflammatory disease. The activation of the pyrin inflammasome is the mainstay of the pathogenesis, which might lead to a specific cell-death mechanism, pyroptosis. Pyroptos...

Clinical Associations of E148Q Heterozygosity: What to Expect From E148Q?

Küçükali B, Bayraktar EÖ, Yıldız Ç , et al. · Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases · 2024

The exact effects of MEFV variants on inflammation are still under investigation, and reports on variants of unknown significance, particularly the E148Q variant, have been conflicting. Therefore, thi...

Perioperative care of the patient with a periodic fever syndrome.

Hanlon CE, Balmuri N, Vanderhoek SM · Journal of clinical anesthesia · 2024

Periodic fever syndromes are autoinflammatory disorders associated with recurrent fevers unrelated to infection. Little is known about the perioperative management of patients with these syndromes, an...

Effect of Colchicine Treatment on Clinical Course in Children with PFAPA Syndrome.

Bagrul İ, Aydin EA, Tuncez S , et al. · Klinische Padiatrie · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is the most common periodic fever condition in children. There is no consensus on treatment to prevent attacks...

Exploring factors for predicting colchicine responsiveness in children with PFAPA.

Özaslan Z, Şen A, Uçar SA , et al. · European journal of pediatrics · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) are the most common autoinflammatory syndromes in children. This study aimed to evaluate the clinical and labor...

Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.

Vyzhga Y, Wittkowski H, Hentgen V , et al. · Pediatric rheumatology online journal · 2024

Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndro...

COVID 19 vaccination as a trigger of acute genital ulcers in an immunocompromised adolescent-case study and literature review.

Pokora K, Kowalczyk K, Peterek R , et al. · BMC women's health · 2024

Acute genital ulcers can affect females of all ages. In children, they often appear as an emergency and remain a diagnostic challenge for pediatricians, gynecologists and dermatologists. Prompt diagno...

Altered serum antibody levels in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.

Gazi U, Dalkan C, Sanlidag B , et al. · Archives of rheumatology · 2024

This study aimed to extend the literature by analyzing immunoglobulin (Ig) A, IgE, IgG, IgG2, IgG3, and IgM antibody levels in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (...

Variables for differential diagnosis of familial Mediterranean fever: Multiple correspondence analysis of a large Japanese cohort.

Kishida D, Nakamura A, Yazaki M , et al. · Modern rheumatology · 2024

We investigated differential diagnoses that should be noted with familial Mediterranean fever (FMF) and useful variables for differentiation in a large Japanese cohort. Patients aged ≥13 years who wer...

Pyrin Inflammasome Activation Defines Colchicine-Responsive SURF Patients from FMF and Other Recurrent Fevers.

Palmeri S, Penco F, Bertoni A , et al. · Journal of clinical immunology · 2024

Syndrome of undifferentiated recurrent fever (SURF) is characterized by recurrent fevers, a lack of confirmed molecular diagnosis, and a complete or partial response to colchicine. Despite the clinica...

Tonsillotomy for Periodic Fever Syndrome: A Randomized and Controlled Trial.

Lantto U, Tapiainen T, Pokka T , et al. · The Laryngoscope · 2024

Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Tonsillotomy has a milder operative risk profile and postoperative...

Assessment of recurrent fever among children undergoing tonsillectomy.

Espahbodi M, Edwards KM, Goudy SL , et al. · BMC pediatrics · 2024

Recurrent tonsillitis is a common indication for tonsillectomy in children and has phenotypic overlap with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. We...

A Potential Link Between Outcome of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) Syndrome in Children and Breastfeeding: A Retrospective Single-Center Cohort Snapshot.

Rigante D, Candelli M · Children (Basel, Switzerland) · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome, often referred to as PFAPA syndrome, may enigmatically recur for an undetermined time in affected children: a potentia...

Adult-Onset PFAPA Syndrome: Successful Management with Colchicine.

Balagani A, Tariq F, Zaheer M · The American journal of case reports · 2024

BACKGROUND Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory fever syndrome primarily seen in children under age 5 years, and its etiology...

Antibiotic prescriptions to children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis.

Rydenman K, Berg S, Karlsson-Bengtsson A , et al. · Acta paediatrica (Oslo, Norway : 1992) · 2024

To investigate the rate of dispensed antibiotic prescriptions to children and adolescents with PFAPA and compare this with the rate for children in the general population. Furthermore, to compare disp...

The use of oral colchicine as a therapeutic and prophylactic approach in a case of PFAPA syndrome with recurrent genital ulcers: an under-recognized manifestation?

Sekine S, Oyama N, Io K , et al. · European journal of dermatology : EJD · 2024

The evaluation of depression and anxiety levels of mothers of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome and familial Mediterranean fever.

Arslanoglu Aydin E, Baglan E, Bagrul İ , et al. · Postgraduate medicine · 2024

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF) are autoinflammatory disorders typically characterized by recurrent fever attacks....

The effect of vitamin D supplementation on attacks in PFAPA syndrome patients with low vitamin D levels.

Öner N, Çelikel E, Tekin ZE , et al. · Irish journal of medical science · 2024

To evaluate the effect of vitamin D supplementation on the frequency and duration of attacks in patients of PFAPA syndrome with low vitamin D levels. This retrospective study comprised PFAPA patients...

Management of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis with Intracapsular Tonsillectomy.

Ezeh UC, Kahn PJ, April MM · The Laryngoscope · 2024

This study aimed to present 2 children clinically diagnosed with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and treated with intracapsular tonsillectomy with adeno...

Attempts to identify the molecular cause of autoinflammatory recurrent fever.

Boyarchuk O, Savkiv D · Reumatologia · 2024

Systemic autoinflammatory diseases caused by dysregulation of the innate immunity are a known cause of recurrent fevers. We present the molecular diagnosis results of 12 children with recurrent fever,...

Use of Streptococcus Salivarius K12 in a cohort of PFAPA patients.

Spagnolo A, Mileto V, Civino A , et al. · Frontiers in pediatrics · 2024

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is the most common cause of periodic fever in childhood. Although PFAPA is generally a self-limited condition, i...

Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome on the Basis of Selective IgA Deficiency.

Altiner S, Ekinci A · Case reports in dermatological medicine · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) is an autoinflammatory disease that is thought to occur with the contribution of genetic and environmental fact...

Risk of immediate postoperative fever in PFAPA patients undergoing tonsillectomy.

Madgar O, Shaffer AD, Chi DH · American journal of otolaryngology · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. Tonsillectomy is considered a potential treatment optio...

Update on ocular manifestations of the main monogenic and polygenic autoinflammatory diseases.

Fonollosa A, Carreño E, Vitale A , et al. · Frontiers in ophthalmology · 2024

Autoinflammatory diseases include disorders with a genetic cause and also complex syndromes associated to polygenic or multifactorial factors. Eye involvement is present in many of them, with differen...

Disease phenotypes in adult patients with suspected undifferentiated autoinflammatory diseases and PFAPA syndrome: Clinical and therapeutic implications.

Gómez-Caverzaschi V, Yagüe J, Espinosa G , et al. · Autoimmunity reviews · 2024

Undifferentiated autoinflammatory diseases are characterized by recurrent or persistent fever, usually combined with other inflammatory manifestations, and negative or inconclusive genetic studies for...

Case Report: Identification of a CARD8 variant in all three patients with PFAPA syndrome complicated with Kawasaki disease.

Nakamura H, Kikuchi A, Sakai H , et al. · Frontiers in pediatrics · 2024

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA syndrome), and Kawasaki disease (KD) are both considered to be disorders of the innate immune system, and the potential r...

The expanding clinical spectrum of autoinflammatory diseases with variants: a case series and literature review.

Karamanakos A, Vougiouka O, Sapountzi E , et al. · Frontiers in immunology · 2024

To assess the impact conferred by variants on the clinical spectrum of patients with systemic autoinflammatory diseases (SAIDs) in Greece. Consecutive patients (n=167) with confirmed SAIDs who underw...

[Síndrome de fiebre periódica, adenitis, faringitis y aftosis en un preescolar con síndrome de Asperger: reporte de caso].

Montero-Vázquez RA · Revista alergia Mexico (Tecamachalco, Puebla, Mexico : 1993) · 2023

Autoinflammatory diseases are a heterogeneous group of pathologies whose prevalence is undefined to date. Within these, the Periodic Fever Syndrome, Adenitis, Pharyngitis and Aphthosis (PFAPA) is dete...

Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients.

Broide M, Levinsky Y, Tal R , et al. · Pediatric rheumatology online journal · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children; by definition, episodes occur every 2 to 8 weeks. Howev...

Autoinflammatory Diseases/Periodic Fevers.

Schutt C, Siegel DM · Pediatrics in review · 2023

Children with intermittent fevers present to pediatricians and other primary care child health providers for evaluation. Most patients will have self-limited, benign infectious illnesses. However, the...

A Potential Partnership between Genetics and the Oral Microbiome in Children Displaying Periodic Fever/Aphthosis/Pharyngitis/Adenitis Syndrome.

Rigante D, Calò L, Ciavarro A , et al. · International journal of molecular sciences · 2023

Periodic fever/aphthosis/pharyngitis/adenitis (PFAPA) syndrome was initially described in a small cohort of American children [...].

[Marshall syndrome].

Pillet P, Bailhache M · La Revue du praticien · 2023

MARSHALL SYNDROME. Marshall syndrome also known as PFAPA syndrome belongs to the group of autoinflammatory diseases. The acronym reflects the main clinical features of the disease: periodic fever, aph...

NLRP12-associated autoinflammatory disease: much more than the FCAS phenotype.

Demir F, Sözeri B · Clinical and experimental rheumatology · 2023

NLRP12-associated autoinflammatory disease (NLRP12-AID) is a rarely seen periodic fever syndrome also known as familial cold autoinflammatory syndrome-2 (FCAS2), caused by autosomal dominant inherited...

PFAPA syndrome in children.

Gardner NJ · JAAPA : official journal of the American Academy of Physician Assistants · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is, as the name implies, characterized by an extremely regular cycle of fevers that is accompanied by one or more other...

TNFRSF1A-pR92Q variant identifies a subset of patients more similar to systemic undifferentiated recurrent fever than TNF receptor-associated periodic syndrome.

Gerritsma AM, Sutera D, Cantarini L , et al. · Clinical and experimental rheumatology · 2023

To describe the clinical phenotype and response to treatment of autoinflammatory disease (AID) patients with the TNFRSF1A-pR92Q variant compared to patients with tumour necrosis factor receptor-associ...

Evaluation of the Levels of Blood Cells, Vitamin D, and Inflammatory Factors in Children with PFAPA Syndrome.

Abbasi E, Mamizadeh N, Seidkhani H , et al. · Clinical laboratory · 2023

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA syndrome) is the most common type of recurrent fever in childhood. The aim of this study was evaluation o...

What is PFAPA syndrome? Genetic clues about the pathogenesis.

Manthiram K · Current opinion in rheumatology · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in childhood. Recent studies report genetic susceptibility variants...

Dermatologic Manifestations of Noninflammasome-Mediated Autoinflammatory Diseases.

Symmank D, Borst C, Drach M , et al. · JID innovations : skin science from molecules to population health · 2023

Autoinflammatory diseases (AIDs) arise from disturbances that alter interactions of immune cells and tissues. They give rise to prominent (auto)inflammation in the absence of aberrant autoantibodies a...

Can the effectiveness of tonsillectomy for PFAPA syndrome be predicted based on clinical factors.

Hara M, Morimoto N, Watabe T , et al. · International journal of rheumatic diseases · 2023

To evaluate the clinical factors associated with the outcome of tonsillectomy in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, thereby clarify...

Kawasaki Disease and Inborn Errors of Immunity: Exploring the Link and Implications.

Sharma S, Nadig PL, Pilania RK , et al. · Diagnostics (Basel, Switzerland) · 2023

The exact etiopathogenesis of Kawasaki disease (KD), the most common childhood vasculitis, remains unknown; however, an aberrant immune response, possibly triggered by an infectious or environmental a...

Tonsillectomy reduces the family impact of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome and improves health-related quality of life in affected children.

Rydenman K, Sparud-Lundin C, Karlsson-Bengtsson A , et al. · Orphanet journal of rare diseases · 2023

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder that primarily affects young children, and typically gives rise to fever episode...

Utility of a targeted next-generation sequencing-based genetic screening panel in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Kışla Ekinci RM, Anlaş Ö, Özalp Ö · Archives of rheumatology · 2023

This study aims to investigate a genetic panel in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and examine its performance for an accurate differential...

Diagnostic performance of routine blood parameters in periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Onur H, Onur AR · Journal of clinical laboratory analysis · 2023

We aimed to investigate the difference between PFAPA and streptococcal tonsillitis (Strep Pharyngitis) by using blood parameters. We want to evaluate the relationship between periodic fever, aphthous...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome and Syndrome of Unexplained Recurrent Fevers in Children and Adults.

Hausmann J, Dedeoglu F, Broderick L · The journal of allergy and clinical immunology. In practice · 2023

Children and adults with autoinflammatory disorders, who often experience recurrent fevers, rashes, cold-induced symptoms, conjunctivitis, lymphadenopathy, recurrent infections, aphthous stomatitis, a...

Exploring the immunological basis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: immunohistochemical staining features of palatine tonsils.

Türkuçar S, Bülbül G, Ünsal E , et al. · Clinical rheumatology · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever syndrome during early childhood period with regular febrile attacks of sterile upper a...

Hiding in plain sight: how the COVID-19 pandemic unmasked the autoinflammatory PFAPA syndrome lurking in our midst.

Sinnappurajar P, Ramanan AV, Goenka A · Archives of disease in childhood · 2023

Current Evidence on Vaccinations in Pediatric and Adult Patients with Systemic Autoinflammatory Diseases.

Massaro MG, Caldarelli M, Franza L , et al. · Vaccines · 2023

Systemic autoinflammatory diseases (SAIDs) are defined by recurrent febrile attacks associated with protean manifestations involving joints, the gastrointestinal tract, skin, and the central nervous s...

Efficacy of canakinumab in a patient with adult-onset glucocorticoid-resistant periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Sopeña B, Araújo O, Freire M , et al. · Modern rheumatology case reports · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, a polygenic or multifactorial condition, is the most frequent autoinflammatory disease in children. There is i...

Two Siblings With Recurrent Fevers: The Path to Mevalonate Kinase Deficiency Diagnosis.

Pereira-Nunes J, Ferreras C, Grangeia A , et al. · Cureus · 2023

Systemic autoinflammatory diseases (SAIDs) are a group of disorders that constitute a rare cause of recurrent fevers. Recurrent fevers are defined as periodic febrile episodes lasting from days to wee...

Does having MEFV gene sequence variants affect the clinical course and colchicine response in children with PFAPA syndrome?

Otar Yener G, Aktaş İ, Altıntaş Meşe C , et al. · European journal of pediatrics · 2023

The primary aim of this study was to document the treatment modalities used in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and look for the efficacy and safety of c...

Long-term efficacy of tonsillectomy/adenotonsillectomy in patients with periodic fever aphthous stomatitis pharyngitis adenitis syndrome with special emphasis on co-existence of familial Mediterranean fever.

Gozen ED, Yildiz M, Kara S , et al. · Rheumatology international · 2023

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is one of the most common autoinflammatory fever disorders in the childhood which may co-exists with familial Mediterranean...

NLRP3 gene variants and serum NLRP3 levels in periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome.

Kaynak D, Yildiz M, Sahin S , et al. · Clinical rheumatology · 2023

Although most of the autoinfammatory disorders have a confirmed genetic cause, periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome still has an unknown genetic bac...

Haploinsufficiency A20 misdiagnosed as PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome with Kikuchi disease.

Jo KJ, Park SE, Cheon CK , et al. · Clinical and experimental pediatrics · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis recurrence temporally associated with allergen-specific immunotherapy in a female adolescent: a case report.

Granjo Morais C, Martins A, Ganhão S , et al. · Reumatismo · 2023

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in pediatric patients. It is clinically characterized by fever flare...

Gray zone in the spectrum of autoinflammatory diseases: familial Mediterranean fever accompanying periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: single-center experience.

Konte EK, Haslak F, Yildiz M , et al. · European journal of pediatrics · 2023

Despite the advanced knowledge concerning autoinflammatory diseases (AID), more data regarding the optimal treatment options and outcomes of the children who met the criteria of more than one AID are...

Adult-onset Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome Responsive to Colchicine.

Nozu T, Ohhira M, Ishioh M , et al. · Internal medicine (Tokyo, Japan) · 2023

We herein report a rare case of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome that occurred in an 18-year-old man. He visited our hospital with recurrent epis...

The use of ketotifen as long-term anti-inflammatory prophylaxis in children with PFAPA syndrome.

Kapustova L, Banovcin P, Bobcakova A , et al. · Frontiers in immunology · 2023

Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA) is the most frequent periodic fever syndrome in children. Its pathogenesis is still unknown, but some disease-modifying f...

Corrigendum: Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.

Mansfield LM, Lapidus SK, Romero SN , et al. · Frontiers in pediatrics · 2023

[This corrects the article DOI: 10.3389/fped.2023.1240242.].

The many faces of pediatric urticaria.

Sekerel BE, Ilgun Gurel D, Sahiner UM , et al. · Frontiers in allergy · 2023

Urticaria is a common disease that can affect individuals of all age groups, with approximately one-quarter of the population experiencing it at least once in their lifetime. Lesions characterized by...

A Challenging Case of Oral Ulcers and Gastrointestinal Bleeding: Crohn's or Behçet's Disease.

Dantas MAS, Graneiro AL, Cavalcante R , et al. · Case reports in pediatrics · 2023

. Differentiating Crohn's disease (CD) and Behçet's disease (BD) with gastrointestinal (GI) manifestations can be clinically challenging, as current diagnostic criteria are not clear between both cond...

Evaluation of common NLRP3 Q703K variant in pediatric patients with autoinflammatory disease: CAPS and PFAPA.

Kendir-Demirkol Y, Jenny LA, Demir F , et al. · The Turkish journal of pediatrics · 2023

Gain-of-function mutations of the NLR family pyrin domain containing 3 (NLRP3) gene have been implicated in autoinflammatory diseases. The NLRP3 Q703K variant is a common variant associated with Cryop...

Increase in pediatric recurrent fever evaluations during the first year of the COVID-19 pandemic in North America.

Mansfield LM, Lapidus SK, Romero SN , et al. · Frontiers in pediatrics · 2023

The impact of the COVID-19 pandemic on new diagnoses of recurrent fevers and autoinflammatory diseases is largely unknown. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) PFAPA/AID...

Febrile children with breaches in the responses of innate immunity.

Rigante D · Expert review of clinical immunology · 2023

Preliminary data revealing efficacy of K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: A multicenter study from the AIDA Network PFAPA syndrome registry.

La Torre F, Sota J, Insalaco A , et al. · Frontiers in medicine · 2023

To evaluate the potential role of K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims wer...

Colchicine - From rheumatology to the new kid on the block: Coronary syndromes and COVID-19.

Surma S, Basiak M, Romańczyk M , et al. · Cardiology journal · 2023

Colchicine is an effective anti-inflammatory agent used to treat gout, coronary artery disease, viral pericarditis, and familial Mediterranean fever. It has been found to act by preventing the polymer...

Epidemiology and clinical features of PFAPA: a retrospective cohort study of 336 patients in western Sweden.

Rydenman K, Fjeld H, Hätting J , et al. · Pediatric rheumatology online journal · 2022

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is generally regarded as the most common autoinflammatory disease, but the epidemiology of the disease is largel...

Synchronous disease onset and flares in siblings with PFAPA.

Dammeyer KL, Schneider A, April MM , et al. · Pediatric rheumatology online journal · 2022

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a clinical syndrome of unclear etiology. PFAPA has generally been considered a non-hereditary fever syndrome; however...

Autoinflammatory syndromes with coexisting variants in Mediterranean FeVer and other genes: Utility of multiple gene screening and the possible impact of gene dosage.

Karamanakos A, Tektonidou M, Vougiouka O , et al. · Seminars in arthritis and rheumatism · 2022

To assess the possible impact conferred by co-existing variants in MEditerranean FeVer (MEFV) and other genes on systemic autoinflammatory disease (SAID) phenotype. Consecutive patients (n = 42) who u...

Mevalonate kinase deficiency/Hyperimmunoglobulin D syndrome (MVK/HIDS) in a Differential Diagnosis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome and Familial Mediterranean Fever (FMF): A Case Report.

Aktaş B, Gümüş D, Tunalı A , et al. · Turkish archives of pediatrics · 2022

Diagnosing PFAPA during the COVID-19 era: clarity during quarantine.

Fiorito T, Akerman M, Noor A , et al. · Archives of disease in childhood · 2022

Clinical characteristics of 10 children with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Lin F, Wang J, Huang J , et al. · Minerva pediatrics · 2022

The Clinical Chameleon of Autoinflammatory Diseases in Children.

Sangiorgi E, Rigante D · Cells · 2022

The very first line of defense in humans is innate immunity, serving as a critical strongpoint in the regulation of inflammation. Abnormalities of the innate immunity machinery make up a motley group...

PFAPA flares observed during COVID outbreak: can emotional stress trigger PFAPA attacks? A multicenter cohort study.

Levinsky Y, Butbul Aviel Y, Ahmad SA , et al. · Pediatric rheumatology online journal · 2022

It is common knowledge among clinicians who treat PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) patients that emotional stress can trigger PFAPA attacks similarly to other autoinf...

Periodic Fever in Children: Etiology and Diagnostic Challenges.

Espin Diaz PC, Singh K, Kher P , et al. · Cureus · 2022

Periodic fever in children is an autoinflammatory illness with an unknown cause. Symptoms include frequent episodes of fever that are followed by an increase in inflammatory markers. A genetic backgro...

Systemic autoinflammatory diseases in pediatric population.

Marques ML, Cunha IM, Alves S , et al. · Asia Pacific allergy · 2022

Systemic autoinflammatory diseases (SAID) are monogenic and polygenic inherited conditions characterized by dysregulation of the innate immune system. We aimed to characterize the clinical features of...

Increased Frequency of Febrile Seizures in Two Periodic Fever Syndromes: Familial Mediterranean Fever and PFAPA Syndrome.

Kılıç H, Gurup Özen A, Barut K , et al. · Turkish archives of pediatrics · 2022

Our aim in this study is to reveal the frequency of febrile seizures in patients with Familial Mediterranean Fever and Periodic Fever, Aphthous stomatitis, Pharyngitis, cervical Adenitis syndrome and...

A comparative study for the clinical features in children with PFAPA syndrome who were diagnosed before and after the age of five.

Kanık A, Eliaçık K, Kanık ET , et al. · International journal of pediatric otorhinolaryngology · 2022

Probiotic use in the prophylaxis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a retrospective cohort study.

Batu ED, Kaya Akca U, Basaran O , et al. · Rheumatology international · 2022

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoinflammatory recurrent fever syndrome that mainly affects children. Probiotics are currently used to prevent u...

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome (PFAPA) or recurrent urinary tract infections: a case report.

Dormanesh B, Asli M, Daryanavard R , et al. · BMC pediatrics · 2022

Fever is the most frequent reason for medical consultation in children, and makes up 15-25% of all consultations in primary care and emergency departments. In here we report a case of a 13 year-old gi...

Cationic Fluoropolyphosphazenes: Synthesis and Assembly with Heparin as a Pathway to Hemocompatible Nanocoatings.

Marin A, Brito J, Sukhishvili SA , et al. · ACS applied bio materials · 2022

The development of state-of-the-art blood-contacting devices can be advanced through integrating hemocompatibility, durability, and anticoagulant functionalities within engineered nanoscale coatings....

The rs13075270 and rs13092160 polymorphisms of CCR1 and CCR3 genes on oral aphthous-like lesions in PFAPA syndrome.

Li W, Li N, Yang G , et al. · Cellular and molecular biology (Noisy-le-Grand, France) · 2022

Fever is a common symptom of infection in children. Periodic fever syndromes are less common but more complex. One of these Periodic fever syndromes is PFAPA (periodic fever, aphthous stomatitis, phar...

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome: Clinical characteristics and treatment outcomes - a single center study in Japan.

Ohnishi T, Sato S, Uejima Y , et al. · Pediatrics international : official journal of the Japan Pediatric Society · 2022

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease occurring in children. Although PFAPA is the most common periodic fever syndrome...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome: A Single-Center Experience.

Yıldız M, Haslak F, Adrovic A , et al. · Turkish archives of pediatrics · 2022

The purpose of this study is to share our experience about clinical findings, natural course, and treatment response rates of a large cohort of patients with periodic fever, aphthous stomatitis, phary...

Galectin-3: a new biomarker for differentiating periodic fever, adenitis, pharyngitis, aphthous stomatitis (PFAPA) syndrome from familial Mediterranean fever?

Batu ED, Vezir E, Öğüş E , et al. · Rheumatology international · 2022

Differentiating PFAPA (periodic fever, aphthosis, pharyngitis, and adenitis) syndrome from familial Mediterranean fever (FMF) could be challenging in some cases. Galectin-3 is a lectin with regulatory...

Response to: 'Do we need the PFAPA syndrome in adults with non-monogenic periodic fevers?' by Fayand .

Gattorno M, Hofer M, Vanoni F , et al. · Annals of the rheumatic diseases · 2022

Do we need the PFAPA syndrome in adults with non-monogenic periodic fevers?

Fayand A, Hentgen V, Ducharme-Bénard S , et al. · Annals of the rheumatic diseases · 2022

The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders.

Oliveira Mendonça L, Matucci-Cerinic C, Terranova P , et al. · Rheumatology (Oxford, England) · 2022

To test the usefulness of an extended panel of lymphocyte subsets in combination with Oliveira's diagnostic criteria for the identification of autoimmune lymphoproliferative syndrome (ALPS) in childre...

[Autoinflammation-differences between children and adults].

Krusche M, Kallinich T · Zeitschrift fur Rheumatologie · 2022

Autoinflammatory diseases present as multisystemic inflammation and often manifest in early childhood. In contrast, in a few diseases, e.g., the recently described VEXAS (vacuoles, E1 enzyme, X‑linked...

The Association Between Vitamin D Level and PFAPA Syndrome: A Systematic Review.

Faydhi SA, Kanawi HMA, Al-Khatib T , et al. · Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2022

Periodic Fever, Aphthous Ulcers, Pharyngitis and Adenitis (PFAPA) Syndrome's etiology is not well understood. The objective of this study is to explore the association between vitamin D level and PFAP...

Episodic aphthous stomatitis with fever, arthralgias and lymphadenopathy following multiple upper respiratory infections and pertussis in an adult: a reactive mimicker of PFAPA syndrome?

Capella GL · Italian journal of dermatology and venereology · 2022

Colchicine versus cimetidine: the better choice for Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome prophylaxis, and the role of MEFV gene mutations.

Raeeskarami SR, Sadeghi P, Vahedi M , et al. · Pediatric rheumatology online journal · 2022

During childhood, the most common periodic fever is periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. The effective treatment and prevention of febrile attacks...

Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF).

Sutera D, Bustaffa M, Papa R , et al. · Seminars in arthritis and rheumatism · 2022

To describe a homogeneous group of patients with undifferentiated recurrent fevers followed-up in a tertiary referral center for systemic autoinflammatory diseases (SAIDs). Patients with undifferentia...

The role of inflammatory mediators in the pathogenesis of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome.

Purrahman D, Poniatowski ŁA, Wojdasiewicz P , et al. · Molecular biology reports · 2022

As a recurrent disease, periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by episodes of febrile attacks and is often prominent in children unde...

Recurrent Aphthous Stomatitis: An Enigmatic Entity and Sign of Systemic Disease.

Elias ML, Fatahzadeh M, Schwartz RA · Indian journal of dermatology · 2022

Recurrent aphthous stomatitis (RAS) is a common oral disease characterized by intermittent eruptions of painful oral ulcerations. Hippocrates first described aphthous stomatitis with the Greek word m...

COVID-19 Confinement Unmasking PFAPA Syndrome.

Daccache A, Feghali E, Feghali EJ , et al. · Kansas journal of medicine · 2022

Challenges in the diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in developing countries-A decade of experience from North India.

Banday AZ, Joshi V, Arora K , et al. · Frontiers in immunology · 2022

Reports of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome from developing countries are sparse. Recognizing PFAPA is often challenging in these regions due to a higher...

PFAPA Syndrome: Clinical, Laboratory and Therapeutic Features in a Single-Centre Cohort.

Lazea C, Damian L, Vulturar R , et al. · International journal of general medicine · 2022

The aim of this study is to describe a group of Romanian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. This consisted of 39 children diagnosed...

Development and implementation of the AIDA International Registry for patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome.

Della Casa F, Vitale A, Cattalini M , et al. · Frontiers in pediatrics · 2022

Aim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphtho...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome in children: a brief literature review.

Okamoto CT, Chaves HL, Schmitz MJ · Revista paulista de pediatria : orgao oficial da Sociedade de Pediatria de Sao Paulo · 2022

To describe clinical, diagnostic and therapeutic characteristics of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome. Literature review in the PubMed databas...

The Added Value of a Multidisciplinary Clinic for Systemic Autoinflammatory Diseases.

Zinterl C, Costa-Reis P, Esteves IC , et al. · Journal of multidisciplinary healthcare · 2022

Systemic autoinflammatory diseases (SAID) are characterized by inappropriate activation of the innate immune system and include not only monogenic periodic fever syndromes but also multifactorial cond...

Editorial: Hereditary Periodic Fevers and Autoinflammatory Diseases.

Sönmez HE, Sözeri B, Aktay Ayaz N · Frontiers in pediatrics · 2022

Assessment of Congenital Neutropenia in Children: Common Clinical Sceneries and Clues for Management.

Lazzareschi I, Rossi E, Curatola A , et al. · Mediterranean journal of hematology and infectious diseases · 2022

A disparate group of rare hematological diseases characterized by impaired maturation of neutrophil granulocytes defines congenital neutropenias. Neutropenic patients are prone to recurrent infections...

Colchicine - an effective treatment for children with a clinical diagnosis of autoinflammatory diseases without pathogenic gene variants.

Welzel T, Wildermuth AL, Deschner N , et al. · Pediatric rheumatology online journal · 2021

Autoinflammatory diseases (AID) are rare chronic conditions with high disease burden, affecting children and adults. Clinically and genetically confirmed, AID can be effectively treated with targeted...

Lymphadenopathy at the crossroad between immunodeficiency and autoinflammation: An intriguing challenge.

Costagliola G, Consolini R · Clinical and experimental immunology · 2021

Lymphadenopathies can be part of the clinical spectrum of several primary immunodeficiencies, including diseases with immune dysregulation and autoinflammatory disorders, as the clinical expression of...

Atypical familial Mediterranean fever with PFAPA-Like symptoms and psoriasis.

Blasco F, García A Á, Martos MD , et al. · Reumatologia clinica · 2021

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome: an overview of genetic background.

Asna Ashari K, Rezaei N · Clinical rheumatology · 2021

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disorder with an uncertain origin. PFAPA manifestations occur in the form of regular att...

Syndrome of Undifferentiated Recurrent Fever (SURF): An Emerging Group of Autoinflammatory Recurrent Fevers.

Papa R, Penco F, Volpi S , et al. · Journal of clinical medicine · 2021

Syndrome of undifferentiated recurrent fever (SURF) is a heterogeneous group of autoinflammatory diseases (AID) characterized by self-limiting episodes of systemic inflammation without a confirmed mol...

Altered tonsillar toll-like receptor (TLR)-1 and TLR-2 expression levels between periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA), and group A beta-hemolytic streptococcal (GAβHS) recurrent tonsillitis patients.

Gazi U, Ozkayalar H, Mujahed MIM , et al. · International journal of pediatric otorhinolaryngology · 2021

Tonsillar microenvironment is thought to contribute to innate immune dysregulation responsible for the periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) because of benefi...

Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.

Luu I, Nation J, Page N , et al. · Clinical immunology (Orlando, Fla.) · 2021

Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined heredita...

Independent risk factors for resolution of periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome within 4 years after the disease onset.

Yildiz M, Haslak F, Adrovic A , et al. · Clinical rheumatology · 2021

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is a polygenic disease with unknown etiology. In this retrospective cohort study, we aimed to evaluate the risk factors...

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: A review.

Wang A, Manthiram K, Dedeoglu F , et al. · World journal of otorhinolaryngology - head and neck surgery · 2021

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common periodic fever condition in children, with most cases appearing by the age of 5. Although PFAPA is ge...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: Relapse and Tonsillar Regrowth After Childhood Tonsillectomy.

Lantto U, Koivunen P, Tapiainen T , et al. · The Laryngoscope · 2021

Tonsillectomy is an effective treatment for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome, but the role of adenoidectomy, as well as later tonsillar regrowth...

Suboptimal vaccination coverage of recommended vaccines among French children with recurrent autoinflammatory fever syndromes: a study from the Juvenile Inflammatory Rheumatism cohort.

Rollet-Cohen V, Mirete J, Dingulu G , et al. · Clinical rheumatology · 2021

To determine vaccination coverage among a French cohort of children with recurrent autoinflammatory fever syndromes (RFS). All RFS children aged 2 to 19 years from the Juvenile Inflammatory Rheumatism...

Clinical Usefulness of Acute-Phase Markers in Distinguishing between PFAPA and Other Exudative Tonsillitis Causes: A Methodological Study.

Kanik A, Eliacik K, Kanik ET , et al. · Journal of tropical pediatrics · 2021

We investigated the practical use of procalcitonin (PCT), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and complete blood count (CBC) parameters in distinguishing periodic fever, aph...

Performance of the new Eurofever/PRINTO classification criteria in Familial Mediterranean fever patients with a single exon 10 mutation in childhood.

Aydın F, Kurt T, Sezer M , et al. · Rheumatology international · 2021

The diagnosis of Familial Mediterranean fever (FMF) based on clinical findings supported by genetic mutation. Recently, the new Eurofever/PRINTO classification criteria including genetic analysis were...

Transcriptome analysis reveals two distinct endotypes and putative immune pathways in tonsils from children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Hara M, Morimoto N, Suzuki N , et al. · Allergy · 2021

IL-1β blockade in periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: case-based review.

Soylu A, Yıldız G, Torun Bayram M , et al. · Rheumatology international · 2021

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome and familial Mediterranean fever (FMF) are considered as inflammasome disorders associated with uncontrolled in...

[Clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: an analysis of 13 cases].

Huang JQ, Ye XH, Yang KK , et al. · Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2021

To study the clinical features of children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, a polygenic and multifactorial autoinflammatory disease with unknown pa...

Children and Adults with PFAPA Syndrome: Similarities and Divergences in a Real-Life Clinical Setting.

Sicignano LL, Rigante D, Moccaldi B , et al. · Advances in therapy · 2021

Analogies or differences of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in children and adults are barely known. The aim of our study was to compare the overall cha...

[What is confirmed in the treatment of autoinflammatory fever diseases?].

Pankow A, Feist E, Baumann U , et al. · Der Internist · 2021

In the last 20 years the clarification of monogenic periodic febrile diseases has led to the independent concept of autoinflammation. In this heterogeneous group polygenic complex diseases are also no...

Rise in children presenting with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome during the COVID-19 pandemic.

Ng KF, Morgan J, Cutts T , et al. · Archives of disease in childhood · 2021

Lingual Tonsillectomy: Successful Treatment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome.

Freeman C, Hines B, Hines J , et al. · Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases · 2021

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is diagnosed clinically. Adult-onset PFAPA syndrome is rare and often has a more diverse clinical presentation...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Rare Etiology of Fever in Adults.

Ferreira Cristina S, Costa A, Toscano M , et al. · Cureus · 2021

We report the case of a 28-year-old male, with a past history of recurrent pharyngitis and tonsillectomy, who presented to the emergency department with fever, pharyngitis, and cervical adenitis. Infl...

The role of Mediterranean fever gene variants in patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Yildiz M, Adrovic A, Ulkersoy I , et al. · European journal of pediatrics · 2021

This study was conducted to investigate the relationship between clinic features and Mediterranean fever gene (MEFV) variants in patients with periodic fever, aphthous stomatitis, pharyngitis, and ade...

Colchicine treatment in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: A multicenter study in Spain.

Quintana-Ortega C, Seoane-Reula E, Fernández L , et al. · European journal of rheumatology · 2021

To evaluate the efficacy of colchicine therapy in pediatric patients with PFAPA syndrome who present with an incomplete response to the standard treatment or with frequent episodes (an interval of les...

Familial Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome; Is It a Separate Disease?

Veres T, Amarilyo G, Abu Ahmad S , et al. · Frontiers in pediatrics · 2021

Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) is the most common periodic fever syndrome in the pediatric population, yet its pathogenesis is unknown. PFAPA was believed to be spo...

Colchicine Effectiveness and Safety in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis.

Welzel T, Ellinghaus M, Wildermuth AL , et al. · Frontiers in pediatrics · 2021

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common fever syndrome in childhood. High disease activity (DA) dramatically impacts the health-related quali...

Children with systemic autoinflammatory diseases have multiple, mixed ethnicities that reflect regional ethnic diversity.

Tucker LB, Niemietz I, Mangat P , et al. · Clinical and experimental rheumatology · 2021

To evaluate the ethnic diversity of children with a systemic autoinflammatory disease (SAID) in a multi-ethnic Canadian province. Self-reported ethnicity of 149 children and adolescents with a SAID in...

How to classify PFAPA? No hard evidence for associated CAPS or CARD variants and not any for links with Behçet's syndrome.

Ben-Chetrit E, Yazici H · Clinical and experimental rheumatology · 2021

Assessment of sleep in children with periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome.

Nalbantoğlu A, Nalbantoğlu B · The Turkish journal of pediatrics · 2021

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common cause of periodic fever in childhood. This study aimed to investigate sleep patterns and possible fact...

Differential cytokine profiles in pediatric patients with PFAPA syndrome and recurrent tonsillitis.

Nakano S, Kondo E, Iwasaki H , et al. · The journal of medical investigation : JMI · 2021

Objective : An attempt was made to identify characteristic cytokine profiles to distinguish periodic fever with aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPAS) from recurrent...

Spectrum of Systemic Auto-Inflammatory Diseases in India: A Multi-Centric Experience.

Suri D, Rawat A, Jindal AK , et al. · Frontiers in immunology · 2021

Systemic autoinflammatory diseases (SAID) are rare inherited disorders involving genes regulating innate immune signaling and are characterized by periodic or chronic multi-systemic inflammation. To...

Vestibular involvement of PFAPA syndrome.

Kuzu S, Günebakan Ç, Yıldız E , et al. · American journal of otolaryngology · 2021

PFAPA syndrome is derived from the initials of the English words of the findings that make up the syndrome ("Periodic Fever", "Aphthous Stomatitis", "Pharyngitis", "Adenitis"). This study aims to eval...

Value of colchicine as treatment for recurrent oral ulcers: A systematic review.

Cabras M, Carrozzo M, Gambino A , et al. · Journal of oral pathology & medicine : official publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology · 2020

In oral medicine, colchicine is a therapeutic alternative for idiopathic recurrent aphthous stomatitis (RAS), Behçet disease (BD), periodic fever, aphthous stomatitis, pharyngitis and cervical adeniti...

Pre- and postoperative quality of life and emotional/behavioural problems in children with PFAPA.

Karayağmurlu A, Aytaç İ · International journal of pediatric otorhinolaryngology · 2020

This study aimed to evaluate the effects of surgical intervention on quality of life, emotional/behavioural problems and school absenteeism in children with periodic fever, aphthous stomatitis, pharyn...

Validation of the new classification criteria for hereditary recurrent fever in an independent cohort: experience from the JIR Cohort Database.

Dingulu G, Georgin-Lavialle S, Koné-Paut I , et al. · Rheumatology (Oxford, England) · 2020

The new classification criteria for the hereditary recurrent fever (HRF) syndrome [cryopyrin-associated periodic syndrome (CAPS), TNF-α receptor-associated periodic syndrome (TRAPS), FMF and mevalonat...

The systemic autoinflammatory diseases: Coming of age with the human genome.

Stone DL, Beck DB, Manthiram K , et al. · The Journal of allergy and clinical immunology · 2020

Diagnostic Rate of Autoinflammatory Diseases Evaluated by Fever Patterns in Pediatric- and Adult-Onset Patients.

Miyamae T, Hanaya A, Kawamoto M , et al. · Journal of clinical rheumatology : practical reports on rheumatic & musculoskeletal diseases · 2020

This is a noncomparative study performed to determine if fever pattern is related to a diagnosis of autoinflammatory disease (AID) in pediatric- and adult-onset patients. The final diagnosis of patien...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome: A debate about diagnosis and treatment in children continues.

Rigante D, Corina L · International journal of pediatric otorhinolaryngology · 2020

The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review.

Førsvoll J, Kristoffersen EK, Øymar K · International journal of pediatric otorhinolaryngology · 2020

Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) s...

Common genetic susceptibility loci link PFAPA syndrome, Behçet's disease, and recurrent aphthous stomatitis.

Manthiram K, Preite S, Dedeoglu F , et al. · Proceedings of the National Academy of Sciences of the United States of America · 2020

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. The disease appears to cluster in families, but the pat...

Why and How Should We Treat Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome?

Hofer M · Paediatric drugs · 2020

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by recurrent fever flares accompanied by symptoms and signs forming the disease acronym and al...

Proposal for a new therapeutic high dosage of Pidotimod in children with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome: a randomized controlled study.

Manti S, Filosco F, Parisi GF , et al. · Italian journal of pediatrics · 2020

Despite to PFAPA syndrome is considered a benign and self-limited condition in childhood its impact on patients and families can be remarkable in many cases. Currently, the therapeutic options for man...

Immune Dysregulation in the Tonsillar Microenvironment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

Luu I, Sharma A, Guaderrama M , et al. · Journal of clinical immunology · 2020

Periodic Fever, Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome is an inflammatory disorder of childhood classically characterized by recurrent fevers, pharyngitis, stomatitis, cervical...

Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG).

Al-Mayouf SM, Almutairi A, Albrawi S , et al. · Rheumatology international · 2020

To define the spectrum and phenotypic characteristics of systemic autoinflammatory diseases (SAIDs) other than familial Mediterranean fever (FMF) in Arab children and to delineate diagnostic evaluatio...

Performance of recently proposed periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome criteria in a region endemic for familial Mediterranean fever.

Adrovic A, Yıldız M, Kanber M , et al. · Rheumatology international · 2020

The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an auto-inflammatory condition characterized by recurrent episodes of fever accompanied by aphthosis, ce...

Performance of the new 'Eurofever/PRINTO classification criteria' in FMF patients.

Sag E, Demirel D, Demir S , et al. · Seminars in arthritis and rheumatism · 2020

Recently a new set of criteria proposed for the classification of auto inflammatory recurrent fevers including familial Mediterranean Fever (FMF). We aimed to compare the sensitivity and specificity o...

Next generation sequencing panel in undifferentiated autoinflammatory diseases identifies patients with colchicine-responder recurrent fevers.

Papa R, Rusmini M, Volpi S , et al. · Rheumatology (Oxford, England) · 2020

The number of innate immune system disorders classified as systemic autoinflammatory diseases (SAID) has increased in recent years. More than 70% of patients with clinical manifestations of SAID did n...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome - Is It Related to Ethnicity? An Israeli Multicenter Cohort Study.

Amarilyo G, Harel L, Abu Ahmad S , et al. · The Journal of pediatrics · 2020

To evaluate the ethnic distribution of Israeli patients with the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA). The medical records of patients with PFAPA attendin...

AA amyloidosis revealing mevalonate kinase deficiency: A report of 20 cases including two new French cases and a comprehensive review of literature.

Rodrigues F, Philit JB, Giurgea I , et al. · Seminars in arthritis and rheumatism · 2020

Mevalonate kinase deficiency (MKD) is a rare autosomal recessive autoinflammatory disease that can lead to an inflammatory A amyloidosis (AA). To study the occurrence of AA in MKD patients we performe...

Long-term management of patients with PFAPA syndrome.

Yıldız E, Kuzu S, Kahveci OK , et al. · European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery · 2020

To evaluate the long-term treatment results of patients with PFAPA syndrome and to determine their need for tonsillectomy. The clinical characteristics, treatments and long-term results of 16 patients...

Recurrence of Symptoms Associated with Menstruation in a Patient with a History of Periodic Fevers.

Padgett C · Journal of pediatric and adolescent gynecology · 2020

Periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome is a cyclic autoinflammatory disease generally diagnosed in childhood. There have been studies suggesting a relationship bet...

Consensus treatment plans for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA): a framework to evaluate treatment responses from the childhood arthritis and rheumatology research alliance (CARRA) PFAPA work group.

Amarilyo G, Rothman D, Manthiram K , et al. · Pediatric rheumatology online journal · 2020

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children. There is considerable heterogeneity in management strat...

Atypical familial mediterranean fever with PFAPA-Like symptoms and psoriasis.

Blasco F, García A Á, Martos MD , et al. · Reumatologia clinica · 2020

Surgical outcomes and unique histological features of tonsils after tonsillectomy in adults with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Yamahara K, Lee K, Egawa Y , et al. · Auris, nasus, larynx · 2020

Data on the adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome are scarce. European studies reported that unlike pediatric-onset PFAPA, tonsillectomy...

Tonsil Mycobiome in PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) Syndrome: A Case-Control Study.

Tejesvi MV, Tapiainen T, Vänni P , et al. · Frontiers in cellular and infection microbiology · 2020

Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome (PFAPA) is the most common periodic fever syndrome in children with unknown etiology, effectively treated with tonsillectomy. Ear...

and Genes Are Differentially Methylated in Patients With Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) Syndrome.

Lovšin E, Kovač J, Tesovnik T , et al. · Frontiers in immunology · 2020

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is the most common autoinflammatory disease in children and is often grouped together with hereditary periodic fever syn...

Current Therapeutic Options for the Main Monogenic Autoinflammatory Diseases and PFAPA Syndrome: Evidence-Based Approach and Proposal of a Practical Guide.

Soriano A, Soriano M, Espinosa G , et al. · Frontiers in immunology · 2020

Monogenic autoinflammatory diseases are rare conditions caused by genetic abnormalities affecting the innate immunity. Previous therapeutic strategies had been mainly based on results from retrospecti...

Increased Prevalence of Q703K Variant Among Patients With Autoinflammatory Diseases: An International Multicentric Study.

Theodoropoulou K, Wittkowski H, Busso N , et al. · Frontiers in immunology · 2020

The inflammasome has been recognized as one of the key components of innate immunity. Gain-of-function mutations in the exon 3 of gene have been implicated in inflammatory diseases suggesting the pr...

The MEFV gene and its association with familial Mediterranean fever, severe atopy, and recurrent respiratory tract infections.

Celiksoy MH, Dogan C, Erturk B , et al. · Allergologia et immunopathologia · 2020

Familial Mediterranean fever (FMF) is the most common auto-inflammatory disease and is characterized by self-limiting episodes of fever and polyserositis. The aim of this study was to determine the at...

Severe Recurrent Fever Episodes With Clinical Diagnosis of Hemophagocytic Lymphohistiocytosis, Incomplete Kawasaki Disease and Systemic-Onset Juvenile Idiopathic Arthritis: A Case Report and Literature Review.

Jiang H, Yang Z · Frontiers in pediatrics · 2020

The pathogeneses of recurrent fever are quite complicated when excluding repeated infections. Recurrent fever is a common symptom for autoinflammatory diseases, relapse of Systemic-onset juvenile idio...

[AN ADULT CASE OF PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS, AND ADENITIS (PFAPA) SYNDROME DEVELOPED IN CHILDHOOD AND DIAGNOSED AFTER PROGRESS MORE THAN 30 YEARS].

Tsukadaira A, Takeuchi Y, Watanabe K , et al. · Arerugi = [Allergy] · 2020

We report an adult case of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, who had a tonsillectomy at 10 years old and relapsed later. An early 40's-year-old man had b...

Rare missense variants in the ALPK1 gene may predispose to periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Sangiorgi E, Azzarà A, Molinario C , et al. · European journal of human genetics : EJHG · 2019

PFAPA is an autoinflammatory syndrome characterized by periodic fever, aphthous stomatitis, sterile pharingitis, and adenitis, with an onset usually before the age of five. While the condition is most...

[PFAPA syndrome - An important differential diagnosis in children with recurrent fever].

Rydenman K, Berg S, Karlsson-Bengtsson A , et al. · Lakartidningen · 2019

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is the most common autoinflammatory disorder among children in many parts of the world and an important differen...

Mediterranean fever gene variants and colchicine therapy in periodic fever, aphthous stomatitis pharyngitis, adenitis syndrome in a Mediterranean region.

Haytoglu Z, Gundeslioglu OO · Expert review of clinical immunology · 2019

Periodic fever, aphthous stomatitis, pharyngitis, adenitis syndrome is characterized by recurrent episodes of fever. Attenuated disease severity was considered in patients with Mediterranean fever (ME...

Recurrence of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome after tonsillectomy: case-based review.

Batu ED, Batu HB · Rheumatology international · 2019

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome for which tonsillectomy is a therapeutic option curing the disease in most patien...

Vitamin D deficiency as a risk factor for PFAPA syndrome.

Nalbantoğlu A, Nalbantoğlu B · International journal of pediatric otorhinolaryngology · 2019

The periodic fever, aphthous stomatitis, pharangytis and cervical adenitis (PFAPA) syndrome, is an idiopathic chronic disease of non-hereditary origin, primarily affecting pre-school children and is t...

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome: main features and an algorithm for clinical practice.

Batu ED · Rheumatology international · 2019

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a recurrent fever syndrome of early childhood with increasing number of adult-onset cases. Although it is a...

Immunogenicity and side-effects of the inactivated hepatitis A vaccine in periodic fever, aphthous stomatitis, pharyngitis, and adenitis patients.

Maritsi DN, Syrmou A, Vartzelis G , et al. · Pediatrics international : official journal of the Japan Pediatric Society · 2019

The aim of this study was to compare the immunogenicity and side-effects of hepatitis A virus (HAV) vaccination between periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) patients...

Treatment options for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome in children and adults: a narrative review.

Gaggiano C, Rigante D, Sota J , et al. · Clinical rheumatology · 2019

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequent non-hereditary autoinflammatory disorder in childhood: Its onset is usually observed befor...

Novel presentations of periodic fever syndromes: Discrepancies between genetic and clinical diagnoses.

Hoang TK, Albert DA · European journal of rheumatology · 2019

The Periodic fever syndromes (PFS) are a group of disorders of the innate immune system. We investigated patients diagnosed with PFS at the Dartmouth Hitchcock Pediatric Rheumatology Clinic. Case acqu...

Incidence of indications for tonsillectomy and frequency of evidence-based surgery: a 12-year retrospective cohort study of primary care electronic records.

Šumilo D, Nichols L, Ryan R , et al. · The British journal of general practice : the journal of the Royal College of General Practitioners · 2019

Neither the incidence of indications for childhood tonsillectomy nor the proportion of tonsillectomies that are evidence-based is known. To determine the incidence of indications for tonsillectomy in...

Familial Mediterranean Fever Is Commonly Diagnosed in Children in Israel with Periodic Fever Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome.

Butbul Aviel Y, Harel L, Abu Rumi M , et al. · The Journal of pediatrics · 2019

To describe a cohort of pediatric patients diagnosed with periodic fever aphthous stomatitis, pharyngitis and adenitis (PFAPA) and familial Mediterranean fever (FMF) and compare them with children dia...

Familial Mediterranean fever and periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: shared features and main differences.

Adrovic A, Sahin S, Barut K , et al. · Rheumatology international · 2019

Autoinflammatory diseases are characterized by fever attacks of varying durations, associated with variety of symptoms including abdominal pain, lymphadenopathy, polyserositis, arthritis, etc. Despite...

Clinical Practice Guideline: Tonsillectomy in Children (Update)-Executive Summary.

Mitchell RB, Archer SM, Ishman SL , et al. · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery · 2019

This update of a 2011 guideline developed by the American Academy of Otolaryngology-Head and Neck Surgery Foundation provides evidence-based recommendations on the pre-, intra-, and postoperative care...

Clinical Practice Guideline: Tonsillectomy in Children (Update).

Mitchell RB, Archer SM, Ishman SL , et al. · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery · 2019

This update of a 2011 guideline developed by the American Academy of Otolaryngology-Head and Neck Surgery Foundation provides evidence-based recommendations on the pre-, intra-, and postoperative care...

Autoinflammatory diseases: State of the art.

Georgin-Lavialle S, Fayand A, Rodrigues F , et al. · Presse medicale (Paris, France : 1983) · 2019

Autoinflammatory diseases are characterized by innate immunity abnormalities. In autoinflammatory diseases (AID), inflammatory blood biomarkers are elevated during crisis without infection and usually...

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome - PFAPA syndrome.

Wekell P · Presse medicale (Paris, France : 1983) · 2019

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a complex autoinflammatory disease with a clinical phenotype characterised by recurrent episodes of fever, s...

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Burton MJ, Pollard AJ, Ramsden JD , et al. · The Cochrane database of systematic reviews · 2019

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a poten...

Phenotypes and genotypes of Chinese adult patients with systemic autoinflammatory diseases.

Hua Y, Wu D, Shen M , et al. · Seminars in arthritis and rheumatism · 2019

We aimed to characterize the phenotypes and genotypes of Chinese adult patients with systemic autoinflammatory diseases (SAIDs). We prospectively evaluated clinical and genetic features of 92 adult pa...

Clinical features and new diagnostic criteria for the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis.

Takeuchi Y, Shigemura T, Kobayashi N , et al. · International journal of rheumatic diseases · 2019

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a common inflammatory disease that presents with periodic fever. We aimed to establish more specific...

Classification criteria for autoinflammatory recurrent fevers.

Gattorno M, Hofer M, Federici S , et al. · Annals of the rheumatic diseases · 2019

Different diagnostic and classification criteria are available for hereditary recurrent fevers (HRF)-familial Mediterranean fever (FMF), tumour necrosis factor receptor-associated periodic fever syndr...

Towards better diagnostic criteria for periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Renko M, Lantto U, Tapiainen T · Acta paediatrica (Oslo, Norway : 1992) · 2019

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of a periodic fever in childhood. The exact pathogenesis and the aetiology of PFAPA are...

From uncertainty to gradually managing and awaiting recovery of a periodic condition- a qualitative study of parents´ experiences of PFAPA syndrome.

Sparud-Lundin C, Berg S, Fasth A , et al. · BMC pediatrics · 2019

The prevalence of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is unknown. Although an uncommon condition, it is considered to be the most common autoinflamm...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome persisting to adulthood - an example of a diagnostic and therapeutic challenge.

Więsik-Szewczyk E, Wolska-Kuśnierz B, Jahnz-Różyk K · Reumatologia · 2019

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most common cause of periodic fever in childhood. Reports of adult patients are sparse. In adults the cli...

A Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome Responsive to Tonsillectomy in Japan.

Yamahara K, Egawa Y, Lee K , et al. · Case reports in otolaryngology · 2019

Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a rare condition, having been reported in only three patients in Japan till date. While almost a...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis in Chinese adult patients.

Wu D, Shen M, Zeng X · Clinical and experimental rheumatology · 2019

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a multifactorial autoinflammatory disease (AID), which mainly affects children. There have been hardly any ca...

The First Case of Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome with Splenomegaly in Iran.

Abolghasemi S, Atashi HA, Paydar-Tali E , et al. · Caspian journal of internal medicine · 2019

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome (PFAPA) is an auto-immune based disease known as a syndrome for pediatrics which typically occurs in children ≤ 5 years of age,...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome (PFAPA): A Clinical Challenge for Primary Care Physicians and Rheumatologists.

Costagliola G, Maiorino G, Consolini R · Frontiers in pediatrics · 2019

To show the different physician's approaches and the difficulties in the diagnosis and management of the Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) syndrome, and t...

Early onset PFAPA-like syndrome in a child with microduplication of the 7q11.23.

Papa R, Dell'Orso G, Caorsi R , et al. · Clinical and experimental rheumatology · 2019

Periodic Fever with Pharyngitis, Aphthous Stomatitis and Cervical Adenitis Syndrome: A Rare Cause of Fever in Adults.

Marques DP, Rocha S, Manso M , et al. · European journal of case reports in internal medicine · 2019

Periodic fever with pharyngitis, aphthous stomatitis and cervical adenitis (PFAPA syndrome) is a common cause of periodic fever in children and usually manifests as episodes of fever recurring with a...

Characteristics and Disease Course in a Cohort of Children With PFAPA Syndrome in the Community of Madrid, Spain.

Ibáñez Alcalde MLM, Caldevilla Asenjo L, Calvo Rey C , et al. · Reumatologia clinica · 2019

PFAPA syndrome is an autoinflammatory disease whose diagnosis is mainly clinical. Several treatments have been proposed; among them, tonsillectomy could be an effective one. Retrospective multicenter...

Long-term follow-up of tonsillectomy efficacy in children with PFAPA syndrome.

Aktas O, Aytuluk HG, Caliskan SK , et al. · Brazilian journal of otorhinolaryngology · 2019

The role of tonsillectomy in the periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, is controversial. Although some studies reported high success rates with tonsillectomy, furthe...

Towards a new set of classification criteria for PFAPA syndrome.

Vanoni F, Caorsi R, Aeby S , et al. · Pediatric rheumatology online journal · 2018

Diagnosis of Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is currently based on the modified Marshall's criteria, but no validated evidence based classificat...

Haploinsufficiency of A20 and other paediatric inflammatory disorders with mucosal involvement.

Aeschlimann FA, Laxer RM · Current opinion in rheumatology · 2018

This review aims at summarizing the current knowledge of A20 haploinsufficiency and other paediatric inflammatory disorders with mucosal involvement. A20 haploinsufficiency is a newly described autoin...

The Influence of Concomitant Disorders on Disease Severity of Familial Mediterranean Fever in Children.

Kişla Ekinci RM, Balci S, Ufuk Altintaş D , et al. · Archives of rheumatology · 2018

This study aims to describe the effects of concomitant disorders on the course of familial Mediterranean fever (FMF) and the relevance of genotype on these associations. Files of 494 FMF patients (257...

Unique histologic features of tonsils from patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Manthiram K, Correa H, Boyd K , et al. · Clinical rheumatology · 2018

The objective of this study is to compare the histology and immune cell composition of tonsils from patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndro...

Pregnancy complicated with PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) syndrome: a case report.

Ota K, Kwak-Kim J, Takahashi T , et al. · BMC pregnancy and childbirth · 2018

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome has been considered as a childhood syndrome. The underlying etiology of PFAPA syndrome is unclear however, curre...

Geoepidemiology and Immunologic Features of Autoinflammatory Diseases: a Comprehensive Review.

Jamilloux Y, Belot A, Magnotti F , et al. · Clinical reviews in allergy & immunology · 2018

The knowledge on systemic autoinflammatory disorders (SAID) is expanding rapidly and new signalling pathways are being decrypted. The concept of autoinflammation has been proposed since 1999, to defin...

[Clinical and genetic analysis of 11 cases with periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome].

Yang Z, He TY, Zhao XD , et al. · Zhonghua er ke za zhi = Chinese journal of pediatrics · 2018

To investigate the clinical, inflammatory and genetic characteristics of cases with periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Clinical and inflammatory manifesta...

Tonsillar antimicrobial peptide (AMP) expression profiles of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) patients.

Gazi U, Agada ME, Ozkayalar H , et al. · International journal of pediatric otorhinolaryngology · 2018

PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most frequent non-infectious cause of high fever observed among the European child population. While its cause is...

PFAPA Syndrome in a Population with Endemic Familial Mediterranean Fever.

Pehlivan E, Adrovic A, Sahin S , et al. · The Journal of pediatrics · 2018

We reviewed the medical records of patients with periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) to investigate the clinical course, treatment response, and association with MEFV gene mut...

Neither hereditary periodic fever nor periodic fever, aphthae, pharingitis, adenitis: Undifferentiated periodic fever in a tertiary pediatric center.

De Pauli S, Lega S, Pastore S , et al. · World journal of clinical pediatrics · 2018

To describe the frequency and clinical characteristics of patients with undifferentiated periodic fever (UPF) and to investigate whether a clinical classification of UPF based on the PRINTO-Eurofever...

High Prevalence of Autoantibodies Against Monomeric C Reactive Protein (CRP) in Children with PFAPA Syndrome.

Kraszewska-Głomba B, Myszka M, Krajewska M , et al. · Journal of medical biochemistry · 2018

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is an autoinflammatory disorder of unknown etiology. The aim of our study was to evaluate whether the presence of a...

Evidence that Enterobius vermicularis plays a causative role for PFAPA and recurrent aphthous stomatitis.

Polese L, Besutti V, Muraro A , et al. · Minerva medica · 2018

Health-related quality of life in children with PFAPA syndrome.

Grimwood C, Kone-Paut I, Piram M , et al. · Orphanet journal of rare diseases · 2018

Conventionally, PFAPA syndrome is considered as a benign disease compared to other recurrent fevers because it completely passes before adulthood. However, in our clinical practice, fever episodes hav...

Risk factors for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a case-control study.

Kettunen S, Lantto U, Koivunen P , et al. · European journal of pediatrics · 2018

The etiology and pathogenesis of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome are unclear. We performed a case-control study to evaluate potential environmental or l...

An international delphi survey for the definition of the variables for the development of new classification criteria for periodic fever aphtous stomatitis pharingitis cervical adenitis (PFAPA).

Vanoni F, Federici S, Antón J , et al. · Pediatric rheumatology online journal · 2018

Diagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall's criteria. Nevertheless no...

Periodic fevers and other autoinflammatory diseases.

Fingerhutová Š, Jančová E, Tesařová M , et al. · Casopis lekaru ceskych · 2018

Autoinflammatory diseases represent a relatively new and rapidly evolving group of rare disorders associated with mutations of genes encoding proteins with a key regulatory role in inflammatory respon...

The Broad-Ranging Panorama of Systemic Autoinflammatory Disorders with Specific Focus on Acute Painful Symptoms and Hematologic Manifestations in Children.

Rigante D · Mediterranean journal of hematology and infectious diseases · 2018

Systemic autoinflammatory disorders (SAIDs) are inherited defects of innate immunity characterized by recurrent sterile inflammatory attacks involving skin, joints, serosal membranes, gastrointestinal...

An Underlooked Cause of Periodic Fever (PFAPA) in an Adult Patient with No Response to Tonsillectomy.

Alam F, Hammoudeh M · Case reports in rheumatology · 2018

Periodic fever with aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is an autoinflammatory disease manifested as recurrent febrile episodes associated with one of the following cardinal feature...

Comorbidity of PFAPA (periodic fever, aphthous stomatitis, pharyngitis and adenitis) patients: a case control study.

Lantto U, Kettunen S, Tapiainen T , et al. · Clinical and experimental rheumatology · 2018

To compare the long-term morbidity of patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome treated by tonsillectomy (TE) in childhood to that of matched contro...

The role of tonsillectomy in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome; a literature review.

Førsvoll J, Øymar K · BMC ear, nose, and throat disorders · 2018

Tonsillectomy (TE) or adenotonsillectomy (ATE) may have a beneficial effect on the clinical course in children with the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis (PFAPA) s...

A patient heterozygous for R92Q mutation with periodic fever and aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome-like phenotype.

Batu ED, Sönmez HE, Bilginer Y , et al. · The Turkish journal of pediatrics · 2018

Batu ED, Sönmez HE, Bilginer Y, Özen S. A patient heterozygous for R92Q mutation with periodic fever and aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome-like phenotype. Turk J Pediatr...

Measles Lymphadenopathy in a Child With PFAPA Syndrome.

Terry J, Brown K, Hiebert J , et al. · Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society · 2018

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is a common cause of periodic fever in children. The pathogenesis of PFAPA is unknown but likely involves immun...

Unraveling the pathogenesis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis through genetic, immunologic, and microbiologic discoveries: an update.

Manthiram K, Lapidus S, Edwards K · Current opinion in rheumatology · 2017

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is considered the most common periodic fever syndrome of childhood. Although it was first described three decad...

A case of adult-onset periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome in Japan.

Yasuura S, Harada Y · Journal of general and family medicine · 2017

Adult-onset periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is rare. We report the case of a 23-year-old woman with PFAPA syndrome in Japan. Her symptoms lasted for app...

Approach to recurrent fever in childhood.

Soon GS, Laxer RM · Canadian family physician Medecin de famille canadien · 2017

To provide an approach to recurrent fever in childhood, explain when infections, malignancies, and immunodeficiencies can be excluded, and describe the features of periodic fever and other autoinflamm...

Microarray analysis of circulating microRNAs in familial Mediterranean fever.

Wada T, Toma T, Matsuda Y , et al. · Modern rheumatology · 2017

Familial Mediterranean fever (FMF) is an autoinflammatory disease caused by mutations in MEFV. Mutations in exon 10 are associated with typical FMF phenotypes, whereas the pathogenic role of variants...

Clinical dose effect and functional consequences of R92Q in two families presenting with a TRAPS/PFAPA-like phenotype.

Grandemange S, Cabasson S, Sarrabay G , et al. · Molecular genetics & genomic medicine · 2017

TNF receptor-associated syndrome (TRAPS) is a dominantly inherited autoinflammatory condition caused by mutations in the gene. The mechanism underlying the variable expressivity of the common variant...

[Tonsillectomy in Cases with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome].

Hara M, Yoshihama K, Komori M , et al. · Nihon Jibiinkoka Gakkai kaiho · 2017

The periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease, characterized, as its name suggests, by periodic fever, aphthous stomatitis...

Periodic Fever with Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome Is Associated with a CARD8 Variant Unable To Bind the NLRP3 Inflammasome.

Cheung MS, Theodoropoulou K, Lugrin J , et al. · Journal of immunology (Baltimore, Md. : 1950) · 2017

Periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is a relatively common autoinflammatory condition that primarily affects children. Although tendencies were reported...

Familial Mediterranean fever with P369S/R408Q exon3 variant in pyrin presenting as symptoms of PFAPA.

Yamagami K, Nakamura T, Nakamura R , et al. · Modern rheumatology · 2017

Familial Mediterranean fever (FMF) can be classified into typical and incomplete/atypical types. Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome-like symptoms have...

[Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome: A study and follow-up of 16 cases].

Muinelo Segade A, Vila Sexto L · Anales de pediatria (Barcelona, Spain : 2003) · 2017

Physicians' perspectives on the diagnosis and management of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Manthiram K, Li SC, Hausmann JS , et al. · Rheumatology international · 2017

To assess the practice patterns of pediatric rheumatology and infectious diseases subspecialists in the diagnosis and treatment of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA...

Is colchicine more effective to prevent periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis episodes in Mediterranean fever gene variants?

Gunes M, Cekic S, Kilic SS · Pediatrics international : official journal of the Japan Pediatric Society · 2017

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is the most frequent repetitive fever syndrome in childhood. It is characterized by fever episodes lasting for a...

Evolving Frontiers in the Treatment of Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) Syndrome.

Rigante D, Gentileschi S, Vitale A , et al. · The Israel Medical Association journal : IMAJ · 2017

Fevers recurring at a nearly predictable rate every 3-8 weeks are the signature symptom of periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome, an acquired autoinflamm...

An Up-to-date Approach to a Patient with a Suspected Autoinflammatory Disease.

Lidar M, Giat E · Rambam Maimonides medical journal · 2017

Autoinflammatory diseases (AID) are characterized by seemingly unprovoked self-limited attacks of fever and systemic inflammation potentially leading to amyloidosis. Familial Mediterranean fever (FMF)...

A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome.

Rigante D, Vitale A, Natale MF , et al. · Clinical rheumatology · 2017

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a mysterious disorder characterized by periodically recurrent fevers, oropharyngeal inflammation, and aden...

Periodic fever syndromes.

Lachmann HJ · Best practice & research. Clinical rheumatology · 2017

Periodic fever syndromes are autoinflammatory diseases. The majority present in infancy or childhood and are characterised by recurrent episodes of fever and systemic inflammation that occur in the ab...

Canakinumab efficacy in refractory adult-onset PFAPA syndrome.

Lopalco G, Rigante D, Vitale A , et al. · International journal of rheumatic diseases · 2017

Diagnostic Criteria for Adult-Onset Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome.

Cantarini L, Vitale A, Sicignano LL , et al. · Frontiers in immunology · 2017

To identify a set of variables that could discriminate patients with adult-onset periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome from subjects with fever of un...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (pfapa) syndrome in children.

Semianchuk VB · Wiadomosci lekarskie (Warsaw, Poland : 1960) · 2017

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome refers to a group of primary immunodeficiencies, namely autoinflammatory diseases. Most pediatricians and otolar...

Family History in Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA) Syndrome.

Manthiram K, Nesbitt E, Morgan T , et al. · Pediatrics · 2016

The goal of this study was to describe family history and inheritance patterns in patients with periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome. We performed a cas...

Characteristics of Patients Referred to a Pediatric Infectious Diseases Clinic With Unexplained Fever.

Statler VA, Marshall GS · Journal of the Pediatric Infectious Diseases Society · 2016

Older case series established diagnostic considerations for children meeting a priori definitions of fever of unknown origin (FUO). No recent study has examined the final diagnoses of children referre...

Review of autoinflammatory diseases, with a special focus on periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome.

Wekell P, Karlsson A, Berg S , et al. · Acta paediatrica (Oslo, Norway : 1992) · 2016

There have been remarkable developments in the field of autoinflammatory diseases over the last 20 years. Research has led to definitions of new conditions, increased understanding of disease mechanis...

Mumps, measles and rubella vaccination in children with PFAPA syndrome.

Kraszewska-Głomba B, Matkowska-Kocjan A, Miśkiewicz K , et al. · Vaccine · 2016

There is no published data regarding immunologic response to vaccinations in children with PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis). The aim of this stud...

Surgery vs medical treatment in the management of PFAPA syndrome: a comparative trial.

Erdogan F, Kulak K, Öztürk O , et al. · Paediatrics and international child health · 2016

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a common disorder in children but there is little or no consensus on its optimal diagnosis and management. To...

Procalcitonin and C-reactive protein-based decision tree model for distinguishing PFAPA flares from acute infections.

Kraszewska-Głomba B, Szymańska-Toczek Z, Szenborn L · Bosnian journal of basic medical sciences · 2016

As no specific laboratory test has been identified, PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) remains a diagnosis of exclusion. We searched for a practical use of...

PFAPA syndrome: a review on treatment and outcome.

Vanoni F, Theodoropoulou K, Hofer M · Pediatric rheumatology online journal · 2016

The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment i...

Tonsillar microbiota in children with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) syndrome.

Tejesvi MV, Uhari M, Tapiainen T , et al. · European journal of clinical microbiology & infectious diseases : official publication of the European Society of Clinical Microbiology · 2016

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a childhood febrile syndrome of unknown origin that is often cured with tonsillectomy. We aimed to compare the bacterial micro...

Proceedings of the 2016 Childhood Arthritis and Rheumatology Research Alliance (CARRA) Scientific Meeting : Toronto, Canada. 14-17 April 2016.

Fotis L, Shaikh N, Baszis K , et al. · Pediatric rheumatology online journal · 2016

P1 Serologic evidence of gut-driven systemic inflammation in juvenile idiopathic arthritis Lampros Fotis, Nur Shaikh, Kevin Baszis, Anthony French, Phillip Tarr P2 Oral health and anti-citrullinated p...

Is colchicine an effective treatment in periodic fever, aphtous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome?

Dusser P, Hentgen V, Neven B , et al. · Joint bone spine · 2016

PFAPA syndrome is the most frequent periodic fever syndrome in non-Mediterranean patients. The pathogenesis is unclear and the treatment is purely symptomatic and not standardized. The aim of this stu...

Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry.

Demirkaya E, Saglam C, Turker T , et al. · The Journal of rheumatology · 2016

Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean...

Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center.

Hernández-Rodríguez J, Ruíz-Ortiz E, Tomé A , et al. · Autoimmunity reviews · 2016

Autoinflammatory diseases (AID) are usually diagnosed during the pediatric age. However, adult-onset disease or diagnosis during adulthood has been occasionally described. To assess the clinical and g...

Could familial Mediterranean fever gene mutations be related to PFAPA syndrome?

Celiksoy MH, Ogur G, Yaman E , et al. · Pediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology · 2016

The cause and pathophysiology of PFAPA syndrome is unknown. The aim of this study was to determine all MEFV gene variants relevant to familial Mediterranean fever in children with PFAPA syndrome. All...

Colchicine as a therapeutic option in periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome.

Butbul Aviel Y, Tatour S, Gershoni Baruch R , et al. · Seminars in arthritis and rheumatism · 2016

To evaluate the efficacy of colchicine in reducing the frequency of attacks in patients with PFAPA. We conducted a 6-month open label, randomized, controlled study among patients with PFAPA who attend...

PFAPA syndrome and Behçet's disease: a comparison of two medical entities based on the clinical interviews performed by three different specialists.

Cantarini L, Vitale A, Bersani G , et al. · Clinical rheumatology · 2016

The pediatric syndrome characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) and adult Behçet's disease share some clinical manifestations and are both polyg...

Tonsillectomy for incomplete PFAPA?

Long SS · The Journal of pediatrics · 2016

Long-Term Outcome of Classic and Incomplete PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis) Syndrome after Tonsillectomy.

Lantto U, Koivunen P, Tapiainen T , et al. · The Journal of pediatrics · 2016

To compare the effectiveness of tonsillectomy and the long-term outcome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome in patients fulfilling the classic diagnostic...

Periodic Fever, Aphthosis, Pharyngitis, and Adenitis Syndrome: Analysis of Patients From Two Geographic Areas.

Batu ED, Kara Eroğlu F, Tsoukas P , et al. · Arthritis care & research · 2016

Periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) syndrome is a periodic fever syndrome of childhood with an unknown etiology. Our aim was to compare the features between PFAPA syndrome pat...

[Evidence-based Indications for Tonsillectomy].

Windfuhr JP · Laryngo- rhino- otologie · 2016

One of the most significant clinical trials, demonstrating the efficacy of tonsillectomy (TE) for recurrent throat infection in severely affected children, was published in 1984. This systematic revie...

Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis.

Theodoropoulou K, Vanoni F, Hofer M · Current rheumatology reports · 2016

PFAPA syndrome represents the most common cause of recurrent fever in children in European populations, and it is characterized by recurrent episodes of high fever, pharyngitis, cervical adenitis, and...

Indications for tonsillectomy stratified by the level of evidence.

Windfuhr JP · GMS current topics in otorhinolaryngology, head and neck surgery · 2016

One of the most significant clinical trials, demonstrating the efficacy of tonsillectomy (TE) for recurrent throat infection in severely affected children, was published in 1984. This systematic revie...

A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report.

Konukbay D, Gattorno M, Yildiz D , et al. · Clinical and experimental rheumatology · 2016

To develop and test a new multidimensional questionnaire for assessment of children with auto-inflammatory disease (AID) such as FMF, PFAPA, HIDS, TRAPS in standard clinical care. The juvenile auto-in...

Demographic, clinical and therapeutic findings in a monocentric cohort of adult patients with suspected PFAPA syndrome.

Vitale A, Orlando I, Lopalco G , et al. · Clinical and experimental rheumatology · 2016

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non-Mendelian autoinflammatory disorder until now considered to be specifically limited to paediatric ag...

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome.

Ali NS, Sartori-Valinotti JC, Bruce AJ · Clinics in dermatology · 2016

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome, the most common periodic disorder of childhood, presents with the cardinal symptoms of periodic fever, aphthous stomati...

Guidelines for the management and treatment of periodic fever syndromes: periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Terreri MT, Bernardo WM, Len CA , et al. · Revista brasileira de reumatologia · 2016

To establish guidelines based on scientific evidence for the management of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. The Guideline was prepared from 5 clinical qu...

Various clinical conditions can mimic Crimean-Congo hemorrhagic fever in pediatric patients in endemic regions.

Kara SS, Kara D, Fettah A · Journal of infection and public health · 2016

Crimean-Congo hemorrhagic fever (CCHF) is a tick-borne disease with high mortality. Many disorders can mimic CCHF. It is important to recognize the condition and to perform differential diagnosis in e...

Response to cimetidine in a 1-year-old child with PFAPA syndrome.

Taniguchi K, Ono N, Sakai T , et al. · The Turkish journal of pediatrics · 2016

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is characterized by periodic febrile attacks recurring regularly every 2 to 8 weeks. Although tonsillectomy is...

The first case of adult-onset PFAPA syndrome in Japan.

Kutsuna S, Ohmagari N, Tanizaki R , et al. · Modern rheumatology · 2016

A 26-year-old woman presented with fever and pharyngitis. She previously experienced four periodic febrile episodes at 30- to 40-day intervals. We suspected periodic fever, aphthous stomatitis, pharyn...

DNA damage and oxidative status in PFAPA syndrome.

Tuğrul S, Doğan R, Kocyigit A , et al. · Auris, nasus, larynx · 2015

PFAPA syndrome is a clinical entity of unknown etiology which presents with periodic episodes of fever, aphthous stomatitis, tonsillitis or pharyngitis, and cervical adenitis. In this study we investi...

Analysis of the genetic basis of periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Di Gioia SA, Bedoni N, von Scheven-Gête A , et al. · Scientific reports · 2015

PFAPA syndrome is the most common autoinflammatory syndrome in children from Western countries. In spite of its strong familial clustering, its genetic basis and inheritance pattern are still unknown....

Polyclonal, newly derived T cells with low expression of inhibitory molecule PD-1 in tonsils define the phenotype of lymphocytes in children with Periodic Fever, Aphtous Stomatitis, Pharyngitis and Adenitis (PFAPA) syndrome.

Dytrych P, Krol P, Kotrova M , et al. · Molecular immunology · 2015

PFAPA syndrome is a benign, recurrent inflammatory disease of childhood. Tonsillectomy is one of the therapeutic options with a yet unexplained biological mechanism. We tested whether specific lymphoc...

Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers.

Federici S, Sormani MP, Ozen S , et al. · Annals of the rheumatic diseases · 2015

The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Medi...

Autoinflammatory syndromes as causes of fever of unknown origin.

Lachmann HJ · Clinical medicine (London, England) · 2015

The systemic autoinflammatory syndromes often present with recurrent fevers. They have proved exceptionally informative about the innate immune system. Although extremely rare, they are important to r...

[Effectiveness of pidotimod in combination with bacterial lysates in the treatment of the pfapa (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome].

Buongiorno A, Pierossi N · Minerva pediatrica · 2015

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome is the most common autoinflammatory syndrome in pediatrics, accepted as an hyperimmune condition. Pidotimod is...

Microbes of the tonsils in PFAPA (Periodic Fever, Aphtous stomatitis, Pharyngitis and Adenitis) syndrome - a possible trigger of febrile episodes.

Lantto U, Koivunen P, Tapiainen T , et al. · APMIS : acta pathologica, microbiologica, et immunologica Scandinavica · 2015

Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adenitis (PFAPA) is a childhood febrile syndrome that is often cured by tonsillectomy (TE). We hypothesized that microbes present in the tonsils ma...

Advances in the genetically complex autoinflammatory diseases.

Ombrello MJ · Seminars in immunopathology · 2015

Monogenic diseases usually demonstrate Mendelian inheritance and are caused by highly penetrant genetic variants of a single gene. In contrast, genetically complex diseases arise from a combination of...

Reduced Number of CD8+ Cells in Tonsillar Germinal Centres in Children with the Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis Syndrome.

Førsvoll J, Janssen EA, Møller I , et al. · Scandinavian journal of immunology · 2015

The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is an autoinflammatory disorder of unknown aetiology. Tonsillectomy may cause a prompt resolution of the...

Treatment of periodic Fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Chesshyre E, Bernatoniene J, Ramanan AV , et al. · The Pediatric infectious disease journal · 2015

PFAPA syndrome: Diagnosis in adulthood.

Hernández Vidal MJ, Trasancos Escura C, Meseguer Noguera R , et al. · Revista clinica espanola · 2015

Genital ulcer as a new clinical clue to PFAPA syndrome.

Scattoni R, Verrotti A, Rinaldi VE , et al. · Clinical and experimental dermatology · 2015

Vaginal ulcers can be associated with a number of different diseases. We describe two girls who presented genital ulcers as a persistent symptom of PFAPA (periodic fever, aphthous stomatitis, pharyngi...

The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research.

Kraszewska-Głomba B, Matkowska-Kocjan A, Szenborn L · Mediators of inflammation · 2015

PFAPA syndrome is a chronic disease that is characterized by recurrent episodes of high fever, aphthous stomatitis, pharyngitis, and cervical adenitis. Knowledge regarding the etiology of PFAPA is lim...

Basic Characteristics of Adults with Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome in Comparison with the Typical Pediatric Expression of Disease.

Cattalini M, Soliani M, Rigante D , et al. · Mediators of inflammation · 2015

Autoinflammatory diseases are caused by inflammasome dysregulation leading to overproduction of proinflammatory cytokines and a pathological delay in the inflammation switching off. The progress of ce...

Clinical features and genetic background of the periodic Fever syndrome with aphthous stomatitis, pharyngitis, and adenitis: a single center longitudinal study of 81 patients.

Perko D, Debeljak M, Toplak N , et al. · Mediators of inflammation · 2015

PFAPA syndrome is the most common autoinflammatory disorder in childhood with unknown etiology. The aim of our study was clinical evaluation of PFAPA patients from a single tertiary care center and to...

Interleukin-1 as a common denominator from autoinflammatory to autoimmune disorders: premises, perils, and perspectives.

Lopalco G, Cantarini L, Vitale A , et al. · Mediators of inflammation · 2015

A complex web of dynamic relationships between innate and adaptive immunity is now evident for many autoinflammatory and autoimmune disorders, the first deriving from abnormal activation of innate imm...

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Burton MJ, Pollard AJ, Ramsden JD , et al. · The Cochrane database of systematic reviews · 2014

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a rare clinical syndrome of unknown cause usually identified in children. Tonsillectomy is considered a poten...

Periodic Fever, Aphthous Stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) Syndrome in Iranian Children First Report of Iranian Periodic Fever and Autoinflammatory Registry (IPFAIR).

Mehregan FF, Ziaee V, Ahmadinejad Z , et al. · Iranian journal of pediatrics · 2014

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a nonhereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. No longt...

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenopathy syndrome is associated with activation of GM-CSF and burst-like expression of IL-8 in peripheral blood.

Ling E, Israeli M, Klein T , et al. · Modern rheumatology · 2014

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenopathy (PFAPA) is an autoinflammatory syndrome characterized by periodic fever with aphthous stomatitis, cervical lymphadenopathy, mya...

The mean platelet volume levels in children with PFAPA syndrome.

Tekin M, Toplu Y, Kahramaner Z , et al. · International journal of pediatric otorhinolaryngology · 2014

To assess whether mean platelet volume (MPV) can be used as a marker in the differential diagnosis of periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome. The leucocyte counts,...

Markedly elevated CD64 expressions on neutrophils and monocytes are useful for diagnosis of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome during flares.

Yamazaki T, Hokibara S, Shigemura T , et al. · Clinical rheumatology · 2014

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most commonly encountered autoinflammatory disease in children, but its pathogenesis and diagnostic biom...

[Tonsillitis and sore throat in childhood].

Stelter K · Laryngo- rhino- otologie · 2014

Surgery of the tonsils is still one of the most frequent procedures during childhood. Due to a series of fatal outcome after hemorrhage in children in Austria in 2006, the standards and indications fo...

Periodic Fever: A Review on Clinical, Management and Guideline for Iranian Patients - Part II.

Ahmadinejad Z, Mansouri S, Ziaee V , et al. · Iranian journal of pediatrics · 2014

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. In the first part of this paper, we presented a guideline for appro...

Vitamin D levels and effects of vitamin D replacement in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Stagi S, Bertini F, Rigante D , et al. · International journal of pediatric otorhinolaryngology · 2014

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease characterized by regularly recurrent fever episodes due to seemingly unprovok...

The enigma of periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome.

Esposito S, Bianchini S, Fattizzo M , et al. · The Pediatric infectious disease journal · 2014

International periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome cohort: description of distinct phenotypes in 301 patients.

Hofer M, Pillet P, Cochard MM , et al. · Rheumatology (Oxford, England) · 2014

The aims of this study were to describe the clinical features of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) and identify distinct phenotypes in a large cohort of pa...

Response to the Letter to the Editor regarding "The mean platelet volume levels in children with PFAPA syndrome".

Tekin M · International journal of pediatric otorhinolaryngology · 2014

Letter to the Editor regarding "Mean platelet volume in patients with PFAPA syndrome: confounding factors should be considered".

Varol E · International journal of pediatric otorhinolaryngology · 2014

Clinical and genetic characterization of Japanese sporadic cases of periodic Fever, aphthous stomatitis, pharyngitis and adenitis syndrome from a single medical center in Japan.

Kubota K, Ohnishi H, Teramoto T , et al. · Journal of clinical immunology · 2014

To investigate clinical presentation, genetic background and cytokine profile of Japanese sporadic cases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome. Nine PFAPA s...

Clinical and genetic profile of children with periodic fever syndromes from a single medical center in South East Michigan.

Chandrakasan S, Chiwane S, Adams M , et al. · Journal of clinical immunology · 2014

To report a cohort of children with periodic fever syndromes (PFS) from Southeast Michigan. A retrospective review of medical records for patients referred for periodic fever over 5 years. Sixty-six p...

Prolonged and recurrent fevers in children.

Marshall GS · The Journal of infection · 2014

Some children referred for prolonged fever are actually not having elevated temperatures; the approach here requires dissection of the history and correction of health misperceptions. Others have well...

PFAPA and 12 Common MEFV Gene Mutations Our Clinical Experience.

Salehzadeh F, Vahedi M, Hosseini-Asl S , et al. · Iranian journal of pediatrics · 2014

Marshall Syndrome or PFAPA is an inflammatory periodic disease characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Although PFAPA is an auto inflammatory disease,...

Periodic Fever: a review on clinical, management and guideline for Iranian patients - part I.

Ahmadinejad Z, Mansori S, Ziaee V , et al. · Iranian journal of pediatrics · 2014

Periodic fever syndromes are a group of diseases characterized by episodes of fever with healthy intervals between febrile episodes. The first manifestation of these disorders are present in childhood...

A practical approach to the diagnosis of autoinflammatory diseases in childhood.

Federici S, Gattorno M · Best practice & research. Clinical rheumatology · 2014

Autoinflammatory diseases are characterized by the presence of chronic or recurrent systemic inflammation secondary to abnormal activation of innate immunity pathways. Many of these diseases have been...

Tonsillitis and sore throat in children.

Stelter K · GMS current topics in otorhinolaryngology, head and neck surgery · 2014

Surgery of the tonsils is still one of the most frequent procedures during childhood. Due to a series of fatal outcomes after hemorrhage in children in Austria in 2006, the standards and indications f...

PFAPA syndrome in adults.

Ortega N, Berenguer M, Garre A , et al. · Revista clinica espanola · 2014

SPAG7 is a candidate gene for the periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome.

Bens S, Zichner T, Stütz AM , et al. · Genes and immunity · 2014

Periodic fever, aphthous stomatitis, pharyngitis and adenopathy (PFAPA) syndrome is an auto-inflammatory disease for which a genetic basis has been postulated. Nevertheless, in contrast to the other p...

Tonsillectomy efficacy in children with PFAPA syndrome is comparable to the standard medical treatment: a long-term observational study.

Vigo G, Martini G, Zoppi S , et al. · Clinical and experimental rheumatology · 2014

Tonsillectomy has recently been suggested as an effective treatment for PFAPA syndrome but little is known about its long-term efficacy. We compared the clinical features and the long-term outcome of...

Elevated levels of CXCL10 in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) during and between febrile episodes; an indication of a persistent activation of the innate immune system.

Førsvoll J, Kristoffersen EK, Oymar K · Pediatric rheumatology online journal · 2013

The Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) is the most common periodic fever syndrome in childhood. Clinically, PFAPA may resemble autoinflammatory dis...

Increased intracellular oxygen radical production in neutrophils during febrile episodes of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Sundqvist M, Wekell P, Osla V , et al. · Arthritis and rheumatism · 2013

Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is an autoinflammatory disease of unknown etiology that primarily affects preschool-aged children. PFAPA syndro...

Treatment of autoinflammatory diseases: results from the Eurofever Registry and a literature review.

Ter Haar N, Lachmann H, Özen S , et al. · Annals of the rheumatic diseases · 2013

To evaluate the response to treatment of autoinflammatory diseases from an international registry and an up-to-date literature review. The response to treatment was studied in a web-based registry in...

PFAPA with facial swelling- a new association?

Khodaghalian B, Tewary KK, Narchi H · Indian journal of pediatrics · 2013

PFAPA (periodic fever, apthous stomatitis, pharyngitis, cervical adenitis) is a rare condition of unknown cause affecting children. Although the exact etiology is unknown, inflammatory, immunological...

Vitamin D linked to PFAPA syndrome.

Mahamid M, Agbaria K, Mahamid A , et al. · International journal of pediatric otorhinolaryngology · 2013

the etiology and pathogenesis of PFAPA is still unknown, the rapid response to corticosteroids treatment suggested an immune mechanism. The role of vitamin D in the development of immune mediated dise...

A case of adult periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome associated with endocapillary proliferative glomerulonephritis.

Cazzato M, Neri R, Possemato N , et al. · Clinical rheumatology · 2013

PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously....

Periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis syndrome is linked to dysregulated monocyte IL-1β production.

Kolly L, Busso N, von Scheven-Gete A , et al. · The Journal of allergy and clinical immunology · 2013

The exact pathogenesis of the pediatric disorder periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis (PFAPA) syndrome is unknown. We hypothesized that PFAPA might be due to dysregulate...

Autoinflammatory diseases in pediatrics.

Hausmann JS, Dedeoglu F · Dermatologic clinics · 2013

Autoinflammatory diseases (AIDs) are characterized by recurrent episodes of systemic and organ-specific inflammation. Many of these diseases share fever as a common presenting feature. Physicians need...

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome and IgA nephropathy.

Sugimoto K, Fujita S, Miyazawa T , et al. · Pediatric nephrology (Berlin, Germany) · 2013

A syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA), as well as immunoglobulin A nephropathy (IgAN), may be caused by autoimmune reactivity nephropathy. A 10-year-old...

Incidence, clinical characteristics and outcome in Norwegian children with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; a population-based study.

Førsvoll J, Kristoffersen EK, Øymar K · Acta paediatrica (Oslo, Norway : 1992) · 2013

To describe the incidence, epidemiology, clinical presentation and clinical outcome of children with the syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) in a...

Treating inflammation by blocking interleukin-1 in humans.

Dinarello CA, van der Meer JW · Seminars in immunology · 2013

IL-1 is a master cytokine of local and systemic inflammation. With the availability of specific IL-1 targeting therapies, a broadening list of diseases has revealed the pathologic role of IL-1-mediate...

Development of Kawasaki disease in a patient with PFAPA.

Ninomiya T, Takada H, Nagatomo Y , et al. · Pediatrics international : official journal of the Japan Pediatric Society · 2013

Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA) is one of the autoinflammatory diseases of unknown etiology characterized by regularly recurrent fever episodes...

Profile of inflammatory mediators in tonsils of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome.

Valenzuela PM, Araya A, Pérez CI , et al. · Clinical rheumatology · 2013

The purpose of this study was to analyze the levels of white blood cells and profile of proinflammatory Th1, Th2, Th17, and T regulatory tissue cytokines in the tonsils of periodic fever, aphthous sto...

Is PFAPA syndrome really a sporadic disorder or is it genetic?

Akelma AZ, Cizmeci MN, Kanburoglu MK , et al. · Medical hypotheses · 2013

Periodic fever syndromes are a group of disorders sharing similar symptoms, characterized primarily by regularly recurring fevers. PFAPA syndrome, one of the members of this group of disorders, is a c...

PFAPA syndrome: clinical characteristics and treatment outcomes in a large single-centre cohort.

Król P, Böhm M, Sula V , et al. · Clinical and experimental rheumatology · 2013

This paper aims to describe clinical and laboratory features and disease outcome in a single-centre cohort of patients with PFAPA syndrome (Periodic Fever, Aphtous stomatitis, Pharyngitis, and Adeniti...

Indications of pediatric tonsillectomy.

Gysin C · ORL; journal for oto-rhino-laryngology and its related specialties · 2013

Possible indications for tonsillectomy include sleep apnea and other obstructive sleep-related breathing disorders, recurrent tonsillitis, peritonsillar abscess, periodic fever, aphthous stomatitis, p...

MEFV Variants in Patients with PFAPA Syndrome in Japan.

Taniuchi S, Nishikomori R, Iharada A , et al. · The open rheumatology journal · 2013

The pathogenesis of PFAPA (periodic fever, aphthous stomatitis, pharyngitis, adenitis) syndrome is unknown as yet. In order to understand whether genes implicated in other auto-inflammatory diseases m...

Different presentations in patients with tumor necrosis factor receptor-associated periodic syndrome mutations: report of two cases.

Celebi-Tayfur A, Bilginer Y, Finetti M , et al. · The Turkish journal of pediatrics · 2013

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominant autoinflammatory disorder caused by mutations in the TNFRSF1A gene encoding the 55-kDa receptor for tumor n...

Long-term surgical outcomes of adenotonsillectomy for PFAPA syndrome.

Licameli G, Lawton M, Kenna M , et al. · Archives of otolaryngology--head & neck surgery · 2012

To evaluate the long-term efficacy of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) syndrome. Prospecti...

Periodic fevers with aphthous stomatitis, pharyngitis, and adenitis (PFAPA).

Vigo G, Zulian F · Autoimmunity reviews · 2012

PFAPA syndrome (acronym of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) is the most common cause of periodic fever in childhood. Nowadays, it is considered part of the wide...

Comparison of conventional and low dose steroid in the treatment of PFAPA syndrome: preliminary study.

Yazgan H, Gültekin E, Yazıcılar O , et al. · International journal of pediatric otorhinolaryngology · 2012

Steroids have been widely used to relief symptoms in the patients with PFAPA syndrome. This study was constructed to show the effectiveness of low-dose steroid therapy in patients diagnosed with PFAPA...

[Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) in adults].

Onderka CE, Ridder GJ · Deutsche medizinische Wochenschrift (1946) · 2012

The PFAPA syndrome is characterized by a regular appearance of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis and is associated with a distinctive poor general condition of the...

Periodic fever associated with aphthous stomatitis, pharyngitis and cervical adenitis.

Kyvsgaard N, Mikkelsen T, Korsholm J , et al. · Danish medical journal · 2012

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome is a non-hereditary idiopathic febrile syndrome belonging to the group of autoinflammatory diseases. PFAPA d...

[PFAPA syndrome: study of 10 cases].

Antón-Martín P, Ortiz Movilla R, Álvarez García A , et al. · Medicina clinica · 2012

«PFAPA syndrome» is an autoinflammatory entity consisting of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Its etiology is unknown although a dysregulation in the control of...

[Diagnosis and treatment of PFAPA syndrome].

Hayashi T · Nihon Jibiinkoka Gakkai kaiho · 2012

Pediatric infectious disease: unusual head and neck infections.

Moffett KS · Oral and maxillofacial surgery clinics of North America · 2012

Infections in children in the head and neck regions are common, leading to frequent use and overuse of antibiotics. This review includes common as well as diverse and unusual infectious diseases, such...

C-reactive protein and procalcitonin during febril attacks in PFAPA syndrome.

Yazgan H, Keleş E, Yazgan Z , et al. · International journal of pediatric otorhinolaryngology · 2012

To assess the levels of procalcitonin (PCT) and C-reactive protein (CRP) in children diagnosed with PFAPA (periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis) during their febrile...

A case of resistant adult-onset periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome responsive to anakinra.

Cantarini L, Vitale A, Galeazzi M , et al. · Clinical and experimental rheumatology · 2012

Diagnosis of PFAPA syndrome applied to a cohort of 17 adults with unexplained recurrent fevers.

Cantarini L, Vitale A, Bartolomei B , et al. · Clinical and experimental rheumatology · 2012

The pathophysiology of PFAPA syndrome, mainly characterised by regularly recurring periodic fevers associated with aphthous stomatitis, pharyngitis and/or lymphadenitis, and mostly occurring in the pa...

A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic.

Alenazi A, Al Sonbul A, Al Jumaah S , et al. · Annals of Saudi medicine · 2012

Published data from Saudi Arabia regarding autoinflammatory diseases are scarce. In this study, we describe the clinical and laboratory features of autoinflammatory diseases in Saudi children. Restros...

[Benefits and importance of tonsillectomy in children and youth with PFAPA syndrome].

Ridder GJ, Onderka CE · Laryngo- rhino- otologie · 2011

PFAPA syndrome is characterized by periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis. Tonsillectomy and adenotonsillectomy are surgical treatment options for this periodic fever s...

Urban legends: recurrent aphthous stomatitis.

Baccaglini L, Lalla RV, Bruce AJ , et al. · Oral diseases · 2011

Recurrent aphthous stomatitis (RAS) is the most common idiopathic intraoral ulcerative disease in the USA. Aphthae typically occur in apparently healthy individuals, although an association with certa...

[Autoinflammatory syndromes/fever syndromes].

Schedel J, Bach B, Kümmerle-Deschner JB , et al. · Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte Gebiete · 2011

Hereditary periodic (fever) syndromes, also called autoinflammatory syndromes, are characterized by relapsing fever and additional manifestations such as skin rashes, mucosal manifestations, or joint...

[Periodic fever, aphthous stomatitis, pharyngitis and adenitis: PFAPA syndrome in Argentina].

Rocco R · Anales de pediatria (Barcelona, Spain : 2003) · 2011

PFAPA syndrome is a benign, non-hereditary condition, of unknown etiology and pathogenesis. There are few reports of it in South America. The purpose of this article is to communicate the experience i...

Skin manifestations in autoinflammatory syndromes.

Braun-Falco M, Ruzicka T · Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2011

Autoinflammatory diseases encompass a group of inflammatory diseases that are non-infectious, non-allergic, non-autoimmune and non-immunodeficient. The term was initially coined for a small group of f...

Tonsillectomy in children with periodic fever with aphthous stomatitis, pharyngitis, and adenitis syndrome.

Garavello W, Pignataro L, Gaini L , et al. · The Journal of pediatrics · 2011

To seek evidence supporting a role for tonsillectomy or adenotonsillectomy in the management of affected children with periodic fever with aphthous stomatitis, pharnygitis, and adenitis (PFAPA) syndro...

Clinical practice guideline: tonsillectomy in children.

Baugh RF, Archer SM, Mitchell RB , et al. · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery · 2011

Tonsillectomy is one of the most common surgical procedures in the United States, with more than 530,000 procedures performed annually in children younger than 15 years. Tonsillectomy is defined as a...

Translational research network and patient registry for auto-inflammatory diseases.

Lainka E, Bielak M, Hilger V , et al. · Rheumatology (Oxford, England) · 2011

Auto-inflammatory diseases (AIDs) are characterized by recurrent self-limiting systemic inflammation. In a multicentre effort, we set out to register genetic, epidemiological and clinical features as...

Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Wurster VM, Carlucci JG, Feder HM , et al. · The Journal of pediatrics · 2011

To assess the long-term outcomes of patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome. Patients enrolled in a PFAPA registry were contacted and sur...

PFAPA syndrome in siblings. Is there a genetic background?

Antón-Martín P, Ortiz Movilla R, Guillén Martín S , et al. · European journal of pediatrics · 2011

"PFAPA syndrome" is an autoinflammatory entity composed of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. There have been many reports of children with the disease, but only...

On the road to discovery in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Brown KL, Wekell P, Karlsson A , et al. · Proceedings of the National Academy of Sciences of the United States of America · 2011

Febrile recurrent respiratory infections or PFAPA syndrome: where is the bias?

Mormile R, Rigante D · Minerva pediatrica · 2011

Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) is a disorder of innate immunity and Th1 activation responsive to IL-1 blockade.

Stojanov S, Lapidus S, Chitkara P , et al. · Proceedings of the National Academy of Sciences of the United States of America · 2011

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) is the most common periodic fever disease in children. However, the pathogenesis is unknown. Using a sys...

Long-term clinical profile of children with the low-penetrance R92Q mutation of the TNFRSF1A gene.

Pelagatti MA, Meini A, Caorsi R , et al. · Arthritis and rheumatism · 2011

To analyze the long-term impact of the R92Q mutation of TNFRSF1A in children with periodic fever, in comparison with children with tumor necrosis factor receptor-associated periodic syndrome (TRAPS) w...

The familial Mediterranean fever gene as a modifier of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome.

Berkun Y, Levy R, Hurwitz A , et al. · Seminars in arthritis and rheumatism · 2011

Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) syndrome is a sporadic disease, characterized by periodic attacks of inflammation. Mutations in the MEFV, the gene associated w...

[Periodic fever, aphthous stomatitis, pharyngitis and adenitis syndrome].

Kusuhara K · Nihon Rinsho Men'eki Gakkai kaishi = Japanese journal of clinical immunology · 2011

Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is a non-hereditary autoinflammatory disease, characterized by relatively regular recurrence of febrile episodes of 3-6 d...

Anti-α-enolase Antibodies in Serum from Pediatric Patients Affected by Inflammatory Diseases: Diagnostic and Pathogenetic Insights.

Pontillo A, Di Toro N, Edomi P , et al. · International journal of rheumatology · 2011

Human glycolytic enzyme α-enolase was associated with human diseases and with inflammation. An ELISA test was developed to measure anti-α-enolase AAE IgG and AAE IgA in the serum from patients affecte...

[Periodic fever with aphthous stomatitis, pharyngitis and adenitis: report of 21 cases].

Sampaio I, Marques JG · Acta medica portuguesa · 2011

PFAPA syndrome (Periodic Fever, Aphthous stomatitis, Pharyngitis, Adenitis) is a benign sporadic syndrome of unknown cause and an important diagnosis to consider in the child with recurrent fever and...

A child with PFAPA syndrome complicated by pityriasis lichenoides et varioliformis acuta.

Iba Y, Sugimoto K, Sakata N , et al. · Pediatric dermatology · 2011

We encountered a boy with periodic fever, aphthous-stomatitis, pharyngitis, adenitis syndrome, complicated by a papular rash representing pityriasis lichenoides et varioliformis acuta. Proinflammatory...

PFAPA syndrome in a young adult with a history of tonsillectomy.

Colotto M, Maranghi M, Durante C , et al. · Internal medicine (Tokyo, Japan) · 2011

Since its clinical definition in 1987, the syndrome called, "periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis" syndrome (PFAPA) has been considered peculiar to pediatric age. In t...

Genital ulcers as an unusual sign of periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy syndrome: a novel symptom?

Lin CM, Wang CC, Lai CC , et al. · Pediatric dermatology · 2011

Periodic fever, aphthous stomatitis, pharyngotonsillitis, cervical adenopathy (PFAPA) syndrome, which is characterized by periodic episodes of high fever, aphthous stomatitis, pharyngitis, and cervica...

Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Burton MJ, Pollard AJ, Ramsden JD · The Cochrane database of systematic reviews · 2010

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome) is a rare clinical syndrome of unknown cause usually identified in children. To assess the efficacy of...

Profile of blood cells and inflammatory mediators in periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome.

Brown KL, Wekell P, Osla V , et al. · BMC pediatrics · 2010

This study aimed to profile levels of blood cells and serum cytokines during afebrile and febrile phases of periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome to advance pa...

Periodic fever, apthous stomatitis, pharyngitis and adenitis syndrome.

Caorsi R, Pelagatti MA, Federici S , et al. · Current opinion in rheumatology · 2010

Periodic fever, apthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common cause of periodic fever of unknown origin in childhood. During the last years a number of studies on la...

[Periodic fever: a description of twelve patients with periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA)].

De Cunto C, Britos M, Eymann A , et al. · Archivos argentinos de pediatria · 2010

PFAPA is a periodic fever syndrome characterized by: fever, aphthous stomatitis, pharyngitis and cervical adenitis. It is one of the autoinflammatory syndromes, but yet of unknown etiology. Our aim is...

Extracts from The Cochrane Library: Tonsillectomy for periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome (PFAPA).

Kuppersmith RB, Schams ST, Rosenfeld RM · Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery · 2010

Natural killer cell lymphoma in a pediatric patient with inflammatory bowel disease.

Deneau M, Wallentine J, Guthery S , et al. · Pediatrics · 2010

Tumor necrosis factor α (TNF-α) antibody agents are an effective therapy for the treatment of inflammatory bowel disease (IBD); however, because of the potential for immune suppression with these drug...

PFAPA syndrome is not a sporadic disease.

Cochard M, Clet J, Le L , et al. · Rheumatology (Oxford, England) · 2010

To determine whether PFAPA (periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis) patients have a positive family history (FH) for recurrent fever syndromes. For all patients with PF...

PFAPA syndrome in children: A meta-analysis on surgical versus medical treatment.

Peridis S, Pilgrim G, Koudoumnakis E , et al. · International journal of pediatric otorhinolaryngology · 2010

To compare the range of medical and surgical therapies for children with PFAPA syndrome. A literature search was performed using Medline, Embase, Ovid and Cochrane databases for studies between 1987 a...

Autoimmune hepatitis type 2 arising in PFAPA syndrome: coincidences or possible correlations?

Della Corte C, Ranucci G, Tufano M , et al. · Pediatrics · 2010

PFAPA syndrome is a chronic disease classified in the group of autoinflammatory syndromes characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis in young children. Th...

MEFV, TNF1rA, CARD15 and NLRP3 mutation analysis in PFAPA.

Dagan E, Gershoni-Baruch R, Khatib I , et al. · Rheumatology international · 2010

PFAPA is a periodic fever disease, of unknown etiology, characterized by aphthous stomatitis, pharyngitis and cervical adenitis. To inquire whether genes implicated in other auto-inflammatory diseases...

The protean visage of systemic autoinflammatory syndromes: a challenge for inter-professional collaboration.

Rigante D · European review for medical and pharmacological sciences · 2010

Systemic autoinflammatory syndromes are a group of inherited and acquired disorders of the innate immunity characterized by recurrence of seemingly unprovoked febrile attacks of variable duration and...

A clinical review of 105 patients with PFAPA (a periodic fever syndrome).

Feder HM, Salazar JC · Acta paediatrica (Oslo, Norway : 1992) · 2010

We describe the presentations and clinical outcomes of pediatric patients diagnosed with PFAPA (Periodic Fever, Aphthous lesions, Pharyngitis, and cervical Adenitis). The medical records of children w...

Randomized trial of adenotonsillectomy versus expectant treatment in PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome: is the impasse over?

Bharti B, Bharti S · The Journal of pediatrics · 2010

Elevated immunoglobulin D levels in children with PFAPA syndrome.

Kovacs L, Hlavatá A, Baldovič M , et al. · Neuro endocrinology letters · 2010

The periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) syndrome appears to be more common than generally appreciated and should be differentiated from hereditary periodic f...

Surgical outcomes and histology findings after tonsillectomy in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome.

Peridis S, Koudoumnakis E, Theodoridis A , et al. · American journal of otolaryngology · 2010

This study aimed to evaluate (a) specific histologic findings in children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome who had tonsillectomy and (b) to...

Differentiating PFAPA syndrome from monogenic periodic fevers.

Gattorno M, Caorsi R, Meini A , et al. · Pediatrics · 2009

To analyze whether there were clinical differences between genetically positive and negative patients fulfilling periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrom...

Syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) in siblings.

Valenzuela PM, Majerson D, Tapia JL , et al. · Clinical rheumatology · 2009

PFAPA syndrome is characterized by episodes of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. PFAPA syndrome usually begins in children under 5 years old and normally has sel...

[Periodic fever syndrome/autoinflammatory syndrome].

Kötter I, Schedel J, Kümmerle-Deschner JB · Zeitschrift fur Rheumatologie · 2009

Hereditary periodic fever syndromes (autoinflammatory syndromes) are characterised by relapsing fevers and additional manifestations such as skin rashes, mucosal manifestations, and joint pain. Some o...

Two siblings with periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA) syndrome.

Sampaio IC, Rodrigo MJ, Monteiro Marques JG · The Pediatric infectious disease journal · 2009

PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, adenitis) is a benign sporadic syndrome of unknown cause. We report 2 siblings diagnosed with this syndrome. The second case started c...