Batu ED, Kara Eroğlu F, Tsoukas P, Hausmann JS, Bilginer Y , et al.
Arthritis care & research •
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 patients from Turkey and those from the US, and patients with and without MEFV variants, and to test the performance of the Eurofever criteria in excluding other autoinflammatory disorders. Seventy-one children with PFAPA syndrome, followed in Hacettepe University, in Ankara, Turkey, and 60 patients at Boston Children's Hospital in the US were enrolled. MEFV gene-variant analysis was performed in 56 patients with Sanger sequencing. In patients from Turkey, symptom onset was at a younger age, fever attacks were of shorter duration, and pharyngitis was more frequent, whereas adenitis, headache, and nausea/vomiting were less frequent during attacks, when compared to patients from the US (P < 0.05). More patients from the Turkish cohort were classified in the familial Mediterranean fever (FMF) group according to the Eurofever criteria than patients from the US (66.2% versus 10%; P < 0.001). Two patients were diagnosed with FMF after MEFV analysis. Twenty-one patients (37.5%) had a single MEFV variant. No significant differences in phenotype were found between patients with and without MEFV variants. The differences between patients from the Turkish and US cohorts may be due to epigenetic or environmental factors. In addition, the Eurofever FMF criteria may perform better in certain areas, if the weight of ethnic origin parameter or cutoff values were modified.
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. Prospective case series. Tertiary care pediatric hospital. Pediatric patients meeting the criteria for PFAPA syndrome. Tonsillectomy with adenoidectomy. Resolution of PFAPA symptoms. A total of 124 patients (75 boys and 49 girls) underwent adenotonsillectomy from 2004 to 2011 for relief of cyclical fevers due to PFAPA syndrome. Of the 124 patients, 22 did not meet criteria for inclusion in this study because (1) they had less than 6 months of follow-up after surgery or (2) they were unavailable for follow-up; therefore, 102 patients were included in the study. The mean age at the time of surgery was 58 months (range, 18-179 months). The average duration of follow-up after adenotonsillectomy was 43 months (range, 6-98 months). Of 102 patients, 99 had complete resolution of their symptoms immediately after surgery. Our findings showed complete resolution of symptoms in 99 of 102 patients with PFAPA syndrome who were treated surgically. Patients who meet the clinical criteria for PFAPA syndrome should be offered tonsillectomy and adenoidectomy as part of their treatment options.
To assess the benefits of adenotonsillectomy in the treatment of pediatric patients with PFAPA (periodic fever, aphthous ulcers, pharyngitis, and adenitis) syndrome. Prospective case series. Tertiary care pediatric hospital. Pediatric patients meeting criteria for PFAPA syndrome. Tonsillectomy with or without adenoidectomy. Resolution of PFAPA symptoms. Twenty-seven (14 female, 13 male) children with PFAPA syndrome underwent tonsillectomy with or without adenoidectomy from 2004 through 2006. The length of follow-up for all patients ranged from 8 to 41 months. A total of 26 patients experienced a complete resolution of their symptoms. The 1 child who continued to have febrile episodes had fever cycles that were not regular in duration or interval and in hindsight was not likely a patient with PFAPA syndrome. Our findings showed complete resolution of symptoms in 26 of 27 patients with PFAPA syndrome treated surgically. Patients who meet clinical criteria for PFAPA syndrome should be considered for tonsillectomy and adenoidectomy if they do not respond to medical management.
To review the various causes of period fever in childhood, including the syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA), and to examine the value of tonsillectomy in the treatment of PFAPA syndrome. Retrospective case series. Urban and tertiary care referral children's hospital. Two patients who underwent tonsillectomy for presumed recurrent adenotonsillitis were later diagnosed as having PFAPA syndrome. Intervention Tonsillectomy. Frequency of PFAPA symptoms before and after tonsillectomy. No difference was found in the frequency of PFAPA symptoms after tonsillectomy. Although a larger series of patients is required, our initial experience suggests that tonsillectomy is not always beneficial for patients with PFAPA syndrome.