ArticleClinical immunology (Orlando, Fla.)2021
Undifferentiated recurrent fevers in pediatrics are clinically distinct from PFAPA syndrome but retain an IL-1 signature.
Article in Clinical immunology (Orlando, Fla.), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 3 of them syntheses that pooled it.
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Who cites it
20 citing papers in PubMed, 3 syntheses or guidelines pooled it, 28 citations in OpenAlex.
- Undifferentiated autoinflammatory diseases: a systematic review.World journal of pediatrics : WJP · 2026Pooled it
- Inflammatory biomarkers in pediatric PFAPA syndrome: a systematic review.Frontiers in pediatrics · 2026Pooled it
- Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.Frontiers in immunology · 2024Pooled it
- Juvenile Inflammatory Rheumatism Real-Life Clinical Practice Strategies (JIR-CliPS) network: methodology and the development process.Orphanet journal of rare diseases · 2026Article
- Clinical, immunological characterisation and treatment response of patients with syndrome of undifferentiated recurrent fever in Chinese children and adolescents: a single-centre cohort study.Frontiers in immunology · 2026Article
- The etiological spectrum of pediatric FUO and clinical management of PFAPA/SURF: a ten-year retrospective study.Frontiers in immunology · 2026Article
- Syndrome of undifferentiated recurrent fever (SURF): a multicenter real-world experience from Türkiye.Clinical rheumatology · 2025Observational
- Predictive factors for therapeutic response and cluster analysis in syndrome of undifferentiated recurrent fever (SURF).RMD open · 2025Article
- Features Associated with Response to Tonsillectomy in Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome in Children.The Journal of pediatrics · 2025Article
- Clinical, Immunologic, and Genetic Characteristics in Patients With Syndrome of Undifferentiated Recurrent Fevers.Arthritis & rheumatology (Hoboken, N.J.) · 2025Article
- Periodic Fever, Aphthous Stomatitis, Pharyngitis and Adenitis Syndrome: An Update.Children (Basel, Switzerland) · 2025Review
- Exploring the role of NOD2 variants in pediatric undifferentiated recurrent fever: a clinical and functional perspective.Frontiers in immunology · 2025Article
- A Challenging Case of Recurrent Fever.Cureus · 2024Article
- Unravelling the clinical heterogeneity of undefined recurrent fever over time in the European registries on Autoinflammation.Pediatric rheumatology online journal · 2024Article
- Pyrin Inflammasome Activation Defines Colchicine-Responsive SURF Patients from FMF and Other Recurrent Fevers.Journal of clinical immunology · 2024Article
- The riddle of recurrent fever: a clinical approach to pediatric autoinflammatory diseases.Frontiers in pediatrics · 2024Review
- Article
- IL-1 and autoinflammatory disease: biology, pathogenesis and therapeutic targeting.Nature reviews. Rheumatology · 2022Review
- Features predicting colchicine efficacy in treatment of children with undefined systemic autoinflammatory disease: A retrospective cohort study.European journal of rheumatology · 2022Article
- Systemic Autoinflammatory Diseases: A Growing Family of Disorders of Overlapping Immune Dysfunction.Rheumatic diseases clinics of North America · 2022Review
Corrections and comments
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Authors and funding
13 authors at 2 institutions in 1 country.
Funding
Abstract
Autoinflammatory disorders of the innate immune system present with recurrent episodes of inflammation often beginning in early childhood. While there are now more than 30 genetically-defined hereditary fever disorders, many patients lack a clear diagnosis. Many pediatric patients are often grouped with patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome despite failing to meet diagnostic criteria. Here, we categorize these patients as syndrome of undifferentiated recurrent fever (SURF), and identify the unique features which distinguish them from the PFAPA syndrome. SURF patients were more likely to report gastrointestinal symptoms of nausea, vomiting and abdominal pain, and experienced inconsistent responses to on-demand steroid therapy compared to PFAPA patients. For this previously undefined cohort, an optimal course of therapy remains uncertain, with medical and surgical therapies largely driven by parental preference. A subset of patients with SURF underwent tonsillectomy with complete resolution. Flow cytometric evaluation demonstrates leukocytic populations distinct from PFAPA patients, with reduced CD3+ T cell numbers. SURF patient tonsils were predominantly characterized by an IL-1 signature compared to PFAPA, even during the afebrile period. Peripheral blood signatures were similar between groups suggesting that PFAPA and SURF patient tonsils have localized, persistent inflammation, without clinical symptoms. These data suggest that SURF is a heterogenous syndrome on the autoinflammatory disease spectrum.
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