Autoinflammatory & rare systemic diseases
Differentiating Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) and Syndrome of Undifferentiated Recurrent Fever (SURF): A Comparative Study of Clinical Features, Treatment Response, and Remission
Authors' conclusion
Structured classification distinguishes PFAPA and SURF by phenotype and disease trajectory.
Study context
- Population
- Children with recurrent fever syndromes referred to the Autoinflammatory Clinic at The Hospital for Sick Children, 2016–2024
- Participants
- 235 children
- Exposure
- PFAPA vs SURF classified by Eurofever/PRINTO criteria (PFAPA) and empirical indications (SURF)
- PFAPAn = 155reference
- SURFn = 80
- Outcome
- Time to remission
Methodological detailssingle-centre, principal component analysis, log-rank test, Cox proportional hazards regression
- Single-centre
- Principal component analysis
- Log-rank test
- Cox proportional hazards regression
Main finding
As reported in the abstract
HR for remission
0.68
P = 0.04
By group
Observational data: associations, not effects.
Further findings
Clinical phenotype
- PFAPA more commonly had classical symptoms (aphthous ulcers, pharyngitis, cervical lymphadenopathy); SURF characterised by GI and systemic features (abdominal pain, arthralgia, fatigue)
Phenotypic clusters (principal component analysis (PCA))
- Predominant patterns: classic PFAPA cluster and GI-dominant cluster
- 2
Corticosteroid and colchicine response
- Neither corticosteroid nor colchicine response associated with phenotype or remission
Original publication
Differentiating Periodic Fever, Aphthous Stomatitis, Pharyngitis, Cervical Adenitis (PFAPA) and Syndrome of Undifferentiated Recurrent Fever (SURF): A Comparative Study of Clinical Features, Treatment Response, and Remission. J Rheumatol. Published 1 April 2026. doi:10. 3899/ jrheum. 2025-1174
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