Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients
Authors' 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.
Study context
- Population
- Children with PFAPA attending Schneider Children's Medical Center of Israel, March 2014 to April 2021; concomitant FMF excluded
- Participants
- 365 children
- Exposure
- Extreme PFAPA vs non-extreme PFAPA
- ePFAPAn = 47
- Outcome
- Demographic and clinical features of ePFAPA vs nPFAPA
Methodological detailssingle-centre, medical record review
- Single-centre
- Medical record review
Main finding
As reported in the abstract
Age at disease onset, median (IQR)
P < 0.001
Observational data: associations, not effects.
Further findings
Age at diagnosis, median (IQR)
P < 0.001
Colchicine prophylaxis
P < 0.001
- ePFAPA from presentation vs ePFAPA after corticosteroid treatment (subgroups): ePFAPA from presentation 47%
- ePFAPA from presentation vs ePFAPA after corticosteroid treatment (subgroups): 47% of those with ePFAPA
Symptoms and signs during flares
- Did not differ significantly between ePFAPA and nPFAPA
ePFAPA from presentation vs ePFAPA after corticosteroid treatment (subgroups)
- Demographic and clinical characteristics similar between subgroups
Original publication
Extreme Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA): a discrete group of patients. Pediatr Rheumatol Online J. Published 1 September 2023. doi:10.
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