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Open Access 01-12-2023 | Stomatitis Aphthosa | Research article

Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients

Authors: Mor Broide, Yoel Levinsky, Rotem Tal, Liora Harel, Shoval Shoham, Sabreen Abu Ahmad, Yonatan Butbul Aviel, Gil Amarilyo

Published in: Pediatric Rheumatology | Issue 1/2023

Abstract

Objective

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 ePFAPA, including the two groups, and to compare them to patients with non-extreme PFAPA (nPFAPA).

Study design

: 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, the median (interquartile range) age at disease onset 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 ePFAPA from after corticosteroid treatment.

Conclusion

About half the patients categorized with ePFAPA syndrome already had extreme features upon presentation. Patients with ePFAPA compared to nPFAPA presented and were diagnosed at an earlier age.

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Title: Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients
Authors: Mor Broide
Yoel Levinsky
Rotem Tal
Liora Harel
Shoval Shoham
Sabreen Abu Ahmad
Yonatan Butbul Aviel
Gil Amarilyo
Publication date: 01-12-2023
Publisher: BioMed Central
Keywords: Stomatitis Aphthosa
Pharyngitis
Colchicine
Tonsillectomy
Published in: Pediatric Rheumatology / Issue 1/2023
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-023-00880-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients

Abstract

Objective

Study design

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Study design

Results

Conclusion

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