BackgroundThe febrile episodes of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome occur, by definition, every 2 to 8 weeks.However, a subset of our patients appears to experience attacks at an even higher rate of more than twice a month, and was therefore named extreme PFAPA, or ePFAPA group.ObjectivesTo characterize this group both demographically and clinically in order to compare them to the non-extreme PFAPA (nPFAPA) group.MethodsWe retrospectively

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... viewed the medical records of patients with PFAPA that were treated in the Schneider Children's Medical Center of Israel from 3/2014 to 4/2021. Patients with concomitant familial Mediterranean fever were excluded. Thereafter the ePFAPA and nPFAPA groups were compared using Wilcoxon rank sum, Pearson's chi-squared and Fisher's exact tests.Results47 patients (12.9%) out a total of 365 PFAPA patients were included in the ePFAPA group. Compared to the nPFAPA group, ePFAPA patients had earlier disease onset (age 1.7 vs 2.96 years, P<0.001) and diagnosis (age 2.94 vs 5.02 years, P<0.001). Moreover, after initiation of an abortive treatment with corticosteroids, ePFAPA patients had higher increased flares frequency (72% vs 40%, P<0.001) and were treated with colchicine prophylaxis more often (67% vs 26%, P<0.001). Other clinical and demographic aspects were not significantly different between the two groups.ConclusionEPFAPA patients are a subset of patients who have earlier onset and diagnosis of PFAPA, and also increased flares frequently after abortive therapy with steroids. Current study is underway to describe the long-term outcome of this group.References[1]Periodic Fever, Aphthosis, Pharyngitis, and Adenitis Syndrome: Analysis of Patients From Two Geographic AreasBatu E, Kara Eroğlu F, Tsoukas P, Hausmann J, Bilginer Y, Kenna M, Licameli G, Fuhlbrigge R, Özen S, Dedeoğlu FArthritis Care and Research (2016) 68(12)[2]POS1326 FAMILIAL PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA) SYNDROME; IS IT A SEPARATE DISEASE?Butbul YAnnals of the Rheumatic Diseases (2021) 80(Suppl 1)[3]PFAPA syndrome is not a sporadic diseaseCochard M, Clet J, Le L, Pillet P, Onrubia X, Guéron T, Faouzi M, Hofer MRheumatology (2010) 49(10)[4]PFAPA syndrome in children: A meta-analysis on surgical versus medical treatmentPeridis S, Pilgrim G, Koudoumnakis E, Athanasopoulos I, Houlakis M, Parpounas KInternational Journal of Pediatric Otorhinolaryngology[5]The First International Conference on Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis SyndromeHarel L, Hashkes P, Lapidus S, Edwards K, Padeh S, Gattorno M, Marshall GJournal of Pediatrics (2018) 193[6]Long-term follow-up of children with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndromeWurster V, Carlucci J, Feder H, Edwards KJournal of Pediatrics (2011) 159(6)[7]Periodic fever, aphthous stomatitis, pharyngitis, and adenopathy syndrome: Clinical characteristics and outcomePadeh S, Brezniak N, Zemer D, Pras E, Livneh A, Langevitz P, Migdal A, Pras M, Passwell JJournal of Pediatrics (1999) 135(1)[8]Risk factors for periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome: a case-control studyKettunen S, Lantto U, Koivunen P, Tapiainen T, Uhari M, Renko MEuropean Journal of Pediatrics (2018) 177(8)[9]A clinical review of 105 patients with PFAPA (a periodic fever syndrome)Feder H, Salazar JActa Paediatrica, International Journal of PaediatricsDisclosure of InterestsNone declared