Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): A Discrete Group of Patients

Yoel Levinsky¹, Rotem Tal², Liora Harel², Shoval Shoham³, Sabreen Abu Ahmad⁴, Yonatan Butbul Aviel⁵, Gil Amarilyo² and Mor Broide³, ¹Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ²Pediatric rheumatology clinic, Schneider children's medical center of Israel, Petach Tikva, Israel, ³Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁴Ruth Rappaport Children's Hospital, Rambam Health Care, Haifa, Israel, ⁵Rambam Medical center, Haifa, Israel

Meeting: 2023 Pediatric Rheumatology Symposium

Keywords: Autoinflammatory Disease, Fever, Pediatric rheumatology, quality of life, steroids

Session Information

Date: Thursday, March 30, 2023

Title: Posters: Clinical and Therapeutic I

Session Type: Poster Session A

Session Time: 6:00PM-7:00PM

Background/Purpose: 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. However, in a subset of our patients, we noticed a higher frequency of attacks, of less than 2 weeks, which we refer to as extreme PFAPA (ePFAPA). This group consisted of patients who were extreme upon presentation of PFAPA, and those who became extreme after initiation of abortive corticosteroid treatment.

We aimed to characterize demographic and clinical features of these two groups and to compare them to patients with non-extreme PFAPA (nPFAPA).

Methods: The medical records of 365 patients with PFAPA who attended Schneider Children’s Medical Center of Israel from March 2014 to April 2021 were reviewed. Patients with concomitant familial Mediterranean fever were excluded. Characteristics of the ePFAPA (including subgroups) and nPFAPA groups were compared using Wilcoxon rank sum, Pearson’s chi-squared, and Fisher’s exact tests.

Results: Forty-seven patients (12.9%) were identified as having ePFAPA.Among patients with ePFAPA, compared to patients with nPFAPA, disease onset (median (interquartile range)) was earlier: 1.5 years (0.7-2.5) vs 2.5 years (1.5-4.0), P< 0.001; and diagnosis was younger: 2.6 years (2.0-3.6) vs 4.5 years (3.0-6.2), P< 0.001. A higher proportion of patients with ePFAPA than nPFAPA were treated with colchicine prophylaxis (53% vs 19%, P< 0.001), but symptoms and signs during flares did not differ significantly between these groups. Demographic and clinical characteristics were similar between patients with ePFAPA from presentation of PFAPA (22, 47% of those with ePFAPA) and with ePFAPA from after corticosteroid treatment.

Conclusion: About half the patients categorized with ePFAPAsyndrome met the criteria upon presentation with PFAPA.Patients with ePFAPA compared to nPFAPA presented and were diagnosed at an earlier age

Disclosures: Y. Levinsky: None; R. Tal: None; L. Harel: None; S. Shoham: None; S. Abu Ahmad: None; Y. Butbul Aviel: None; G. Amarilyo: None; M. Broide: None.

To cite this abstract in AMA style:

Levinsky Y, Tal R, Harel L, Shoham S, Abu Ahmad S, Butbul Aviel Y, Amarilyo G, Broide M. Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): A Discrete Group of Patients [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 4). https://acrabstracts.org/abstract/extreme-periodic-fever-aphthous-stomatitis-pharyngitis-adenitis-pfapa-a-discrete-group-of-patients/. Accessed .

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