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

J RheumatolPublished Retrospective cohort study

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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