SynthesisFrontiers in pediatrics2026
Inflammatory biomarkers in pediatric PFAPA syndrome: a systematic review.
Synthesis in Frontiers in pediatrics, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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3 authors.
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Abstract
Background: PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) is the most common periodic fever syndrome in childhood, yet remains a diagnosis of exclusion. No recent synthesis has quantitatively evaluated its circulating biomarkers. We aimed to assess the behavior of inflammatory biomarkers during febrile flares vs. asymptomatic intervals and evaluate their ability to distinguish PFAPA from acute infections and other auto inflammatory diseases. Methods: Following PRISMA 2020, PubMed and Scopus were searched for English-language studies (1 January 2018-07 June 2026) including children (0-18 years) with confirmed PFAPA reporting at least one of eleven predefined biomarkers (WBC, neutrophils, lymphocytes, CRP, SAA, serum calprotectin, IL-6, IL-1β, IL-18, TNF-α, IFN- Results: Seven studies (710 children; five countries; two prospective, five retrospective; 2019-2026) were included from 170 records. CRP and SAA increased markedly during flares and normalized between episodes, with SAA showing the greatest dynamic range. However, neither CRP, WBC, neutrophils, nor IL-6 reliably distinguished PFAPA flares from acute infections. IL-1β was consistently undetectable in serum despite the inflammasome-driven pathophysiology. IFN- Conclusions: CRP and SAA are reliable markers of PFAPA activity but lack discriminating value vs. infection. IFN-
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