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Surgical versus medical management of PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis): a systematic review and network meta-analysis
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DOI:10.1186/s12969-026-01242-3.png)
Abstract
En 中文
Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenitis (PFAPA) syndrome is the most common autoinflammatory periodic fever disorder of childhood. This study aimed to compare the effectiveness and safety of surgical and medical treatments for PFAPA using a systematic review and network meta-analysis (NMA). Medline, Embase, and CENTRAL were searched through November 2025. Randomized and non-randomized comparative studies evaluating surgical interventions (tonsillectomy, adenotonsillectomy, tonsillotomy) and medical therapies (corticosteroids, cimetidine, colchicine, and others) in PFAPA were included. The primary outcome was clinical remission or symptom resolution; secondary outcomes included variation with age and duration of disease. Pairwise and network meta-analyses were performed using random-effects models. Risk of bias was assessed using RoB 2 and ROBINS-I, and certainty of evidence was evaluated using GRADE. Sixteen studies involving 2,048 patients were included. In the primary NMA, no statistically significant difference was observed between pooled surgical and medical management (OR 1.10, 95% CI 0.21–5.69), with very high heterogeneity (I² 97.4%). Exploratory comparisons suggested trends favoring some medical therapies over surgery; however, confidence intervals were wide and the certainty of evidence was very low. Bayesian sensitivity analyses and meta-regressions did not identify age or episode duration as meaningful effect modifiers. Current evidence is insufficient to draw reliable comparative conclusions regarding the relative effectiveness of surgical and medical management for PFAPA. Substantial clinical and methodological heterogeneity, sparse comparative data, and very low-certainty evidence limit interpretation of pooled estimates. Well-designed prospective comparative studies with standardized outcomes are needed to inform treatment decisions.
Keywords:
PFAPA
Inflammatory syndrome
Periodic fever
Surgery
Medical management
Journal
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2.3
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221
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