Autoimmune and Inflammatory Disorders Research (47)
Inflammasome and immune disorders (33)
Ocular Diseases and Behçet’s Syndrome (14)
Adolescent and Pediatric Healthcare (11)
Kawasaki Disease and Coronary Complications (10)
Dipasquale RF, Sinopoli P, Mendicino A, Gallizzi R
European journal of pediatrics •
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-free intervals. This systematic review assessed the effectiveness of daily colchicine prophylaxis in pediatric PFAPA. Furthermore, MEFV status association with colchicine efficacy has been explored. PubMed, Scopus, and Web of Science were systematically searched from inception to 6 November 2025 to identify trials and observational studies presenting participants with a diagnosis of PFAPA, colchicine prophylaxis as interventions, and attack frequency/attack-free interval as outcomes. All eligible records identified up to that date were screened. Colchicine reduced attack frequency, lengthened attack-free intervals, and lowered steroid use, with benefits often within 1 month. A short randomized comparison showed similar 3-month efficacy to cimetidine. Adverse events were mostly mild gastrointestinal; discontinuations were uncommon. MEFV variants as predictors of response remain uncertain. Current evidence supports colchicine as an efficacy and generally well-tolerated preventive option. • PFAPA is the most common periodic fever in children. On-demand corticosteroids stop attacks but may shorten symptom-free intervals; preventive options include cimetidine, selective tonsillectomy, and biologics. • Colchicine modulates innate immunity and it is effective in familial Mediterranean fever; MEFV variants occur in a subset of PFAPA, supporting interest in repurposing. • Continuous colchicine reduced attack frequency, prolonged attack-free intervals, and lowered steroid use; adverse events were mostly mild gastrointestinal, with few treatment discontinuations. • Clinical improvement often appeared by about 1 month and stabilized by 3 months; this supports early reassessment to adjust dose or change therapy. MEFV is not clearly associated.
Spagnolo A, Mileto V, Civino A, Maggio MC, Risso P , et al.
Frontiers in pediatrics •
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, it can have negative impact on child's and parents' quality of life and family functioning. Our primary aim was to assess the potential effectiveness of Streptococcus Salivarius K12 (SSK12) in preventing febrile attacks in PFAPA patients. Secondary objectives included evaluating the effectiveness of SSK12 in mitigating the severity of febrile episodes seen as a statistically significant reduction in the episode duration, highest fever temperature reached during fever, in the frequency of each associated symptom, calculated in the six months before and after the start of therapy. A total of 117 patients with PFAPA were evaluated using Marshall's criteria, modified by Thomas et al. and according to Eurofever/PRINTO classification criteria, aged 6 months to 9 years, with a median age at the onset of the disease of 2 years, treated with SSK12, since January 2021 to January 2023. Data were collected retrospectively. Before using SS K12, febrile episodes recurred on average every 26.1 ± 11.5 days, with a febrile episode duration of 4.1 ± 1.4 days. The highest fever temperature during the episode was 39.8 ± 0.7 °C. After six months of SS K12, febrile episodes recurred on average every 70 ± 53,1 days ( value <0.01), the mean lenght of febrile episodes was 3.3 ± 1.6 ( value <0.01) and the highest fever temperature reached during the febrile episode was 39.1 ± 1.1 °C ( value <0.01). We also documented a reduction in the frequency of exudative pharyngotonsillitis present in 72 vs. 103 patients ( value <0.01), oral aphthosis present in 47 vs. 80 patients ( value <0.01), lateral cervical lymphadenopathy in 45 vs. 83 ( value <0.01). Erythematous pharyngotonsillitis decreased in frequency but it was not statistically significant. The results of our study indicate that the use of SS K12 could be beneficial in decreasing febrile episodes related to PFAPA syndrome and its associated symptoms, potentially improving the quality of life in pediatric patients and decreasing the need for additional pharmacological therapies.
Ter Haar N, Lachmann H, Özen S, Woo P, Uziel Y , et al.
Annals of the rheumatic diseases •
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 which clinical information on anonymised patients with autoinflammatory diseases was collected retrospectively as part of the Eurofever initiative. Participating hospitals included paediatric rheumatology centres of the Paediatric Rheumatology International Trial Organisation network and adult centres with a specific interest in autoinflammatory diseases. The following diseases were included: familial Mediterranean fever (FMF), cryopyrin-associated periodic syndromes (CAPS), tumour necrosis factor (TNF)-receptor associated periodic syndrome (TRAPS), mevalonate kinase deficiency (MKD), pyogenic arthritis pustulosis acne (PAPA) syndrome, deficiency of interleukin-1 receptor antagonist (DIRA), NLRP12-related periodic fever and periodic fever aphthosis pharyngitis adenitis (PFAPA) syndrome. Cases were independently validated by experts for each disease. A literature search regarding treatment of the abovementioned diseases was also performed using Medline and Embase. 22 months from the beginning of the enrolment, complete information on 496 validated patients was available. Data from the registry in combination with evidence from the literature confirmed that colchicine is the treatment of choice for FMF and IL-1 blockade for DIRA and CAPS. Corticosteroids on demand probably represent a valid therapeutic strategy for PFAPA, but also for MKD and TRAPS. Patients with poorly controlled MKD, TRAPS, PAPA or FMF may benefit from IL-1 blockade; anti-TNF treatment may represent a possible valuable alternative. In the absence of high-grade evidence, these results could serve as a basis for therapeutic guidelines and to identify candidate drugs for future therapeutic trials.