106 - Extreme PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) - a discrete group of patients with more frequent flares than previously reported.
Monday, April 25, 2022
3:30 PM – 6:00 PM US MT
Poster Number: 106 Publication Number: 106.409
Mor Broide, Schneider Children's Medical Center of Israel, Ramat Hasharon, Tel Aviv, Israel; Yoel Levinsky, Children's Hospital, Givaat Shmuel, Tel Aviv, Israel
Medical Resident Schneider Children's Medical Center of Israel Ramat Hasharon, Tel Aviv, Israel
Background: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in children and, by definition, episodes occur every 2 to 8 weeks. However, we noticed a higher frequency of attacks in a subset of our patients lasting less than 2 weeks (referred as extreme PFAPA – ePFAPA group).
Objective: We aimed to characterize demographic and clinical features of this group and to compare them to non-extreme PFAPA (nPFAPA) patients.
Design/Methods: The medical records of patients with PFAPA attending the Schneider Children's Medical Center of Israel from 3/2014 to 4/2021 were retrospectively reviewed. Patients with concomitant familial Mediterranean fever were excluded. ePFAPA and nPFAPA groups were compared using Wilcoxon rank sum, Pearson's chi-squared and Fisher's exact tests.
Results: Out of 365 total PFAPA patients, 47 patients (12.9%) were identified as having ePFAPA. 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). compared to nPFAPA patients. In addition, ePFAPA patients had even higher increased flares frequency after initiation of an abortive treatment with corticosteroids (72% vs 40%, P < 0.001) and were more likely to be treated with colchicine prophylaxis (67% vs 26%, P < 0.001). No other significant demographic or clinical differences were identified.Conclusion(s): ePFAPA patients are a discrete group of patients with earlier onset and diagnosis of PFAPA and with increased flares frequently after abortive therapy with steroids. Current study is underway to describe the long-term outcome of this group.
