{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T06:24:17Z","timestamp":1786947857395,"version":"build-2736575974"},"reference-count":20,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T00:00:00Z","timestamp":1786924800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Systemic juvenile idiopathic arthritis (sJIA) is a diagnosis of exclusion with nonspecific early manifestations. Misdiagnosis is common due to overlap with infections, autoinflammatory diseases, and malignancies. We aimed to characterize a series of patients initially diagnosed with sJIA who subsequently reclassified following further evaluation were.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively reviewed the clinical records, laboratory findings, genetic analyses, and imaging data of six pediatric patients initially diagnosed with sJIA but later revised to alternative diagnosis after further investigations. These patients were enrolled at Shenzhen Children's Hospital between January 2011 and December 2022.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All six patients initially fulfilled at least one set of sJIA classification criteria. All patients presented with recurrent fever (100%, 6\/6), three had arthritis (50%, 3\/6), and five exhibited rash (83.3%, 5\/6). Elevated inflammatory markers were observed in all patients (100%, 6\/6). The median age at initial sJIA diagnosis was 64 months (IQR, 24\u2013145). The median delay from initial diagnosis to final diagnosis was 9.5 months (IQR, 5\u201312). Three patients (50%, 3\/6) were considered to have refractory sJIA prior to diagnostic revision. Final diagnoses included autoinflammatory diseases (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093), Takayasu arteritis (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), inflammatory myofibroblastic tumor (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091), and suspected congenital hemophagocytic lymphohistiocytosis (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>sJIA is defined by classification criteria and represents a diagnosis of exclusion, requiring long-term dynamic reassessment to distinguish it from other diseases with overlapping clinical features. Early recognition of atypical manifestations and timely comprehensive evaluation are essential to avoid misdiagnosis, particularly in distinguishing monogenic autoinflammatory disorders and malignancies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1885428","type":"journal-article","created":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T05:25:54Z","timestamp":1786944354000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Diagnostic pitfalls in systemic juvenile idiopathic arthritis: insights from 6 misdiagnosed cases"],"prefix":"10.3389","volume":"14","author":[{"given":"Xiaona","family":"Zhu","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, Shenzhen Children\u2019s Hospital Affiliated Shantou University Medical College, Shantou University","place":["Shenzhen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yinyin","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Shenzhen Children\u2019s Hospital Affiliated Shantou University Medical College, Shantou University","place":["Shenzhen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jixin","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Shenzhen Children\u2019s Hospital Affiliated Shantou University Medical College, Shantou University","place":["Shenzhen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Shenzhen Children\u2019s Hospital Affiliated Shantou University Medical College, Shantou University","place":["Shenzhen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tingyan","family":"He","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Shenzhen Children\u2019s Hospital Affiliated Shantou University Medical College, Shantou University","place":["Shenzhen, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,8,17]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1056\/NEJMra2402073","article-title":"Juvenile idiopathic arthritis","volume":"393","author":"Sandborg","year":"2025","journal-title":"N Engl J Med"},{"key":"B2","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1038\/s41572-021-00332-8","article-title":"Juvenile idiopathic arthritis","volume":"8","author":"Martini","year":"2022","journal-title":"Nat Rev Dis Primers"},{"key":"B3","doi-asserted-by":"publisher","first-page":"13","DOI":"10.1111\/pai.13158","article-title":"Macrophage activation syndrome in pediatrics","volume":"31","author":"Alongi","year":"2020","journal-title":"Pediatr Allergy Immunol"},{"key":"B4","doi-asserted-by":"publisher","first-page":"3045","DOI":"10.1093\/rheumatology\/keab342","article-title":"The IL-18\/IFNgamma axis in systemic JIA and MAS-new answers, more questions","volume":"60","author":"Schulert","year":"2021","journal-title":"Rheumatology"},{"key":"B5","first-page":"390","article-title":"International league of associations for rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001","volume":"31","author":"Petty","year":"2004","journal-title":"J Rheumatol"},{"key":"B6","doi-asserted-by":"publisher","first-page":"190","DOI":"10.3899\/jrheum.180168","article-title":"Toward new classification criteria for juvenile idiopathic arthritis: first steps, pediatric rheumatology international trials organization international consensus","volume":"46","author":"Martini","year":"2019","journal-title":"J Rheumatol"},{"key":"B7","doi-asserted-by":"publisher","first-page":"1614","DOI":"10.1136\/ard-2024-225851","article-title":"EULAR\/PReS recommendations for the diagnosis and management of still\u2019s disease, comprising systemic juvenile idiopathic arthritis and adult-onset still\u2019s disease","volume":"83","author":"Fautrel","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"B8","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1186\/s12969-019-0328-3","article-title":"An unusual presentation of purine nucleoside phosphorylase deficiency mimicking systemic juvenile idiopathic arthritis complicated by macrophage activation syndrome","volume":"17","author":"Arduini","year":"2019","journal-title":"Pediatr Rheumatol Online J"},{"key":"B9","doi-asserted-by":"publisher","first-page":"414","DOI":"10.1080\/03009742.2022.2049043","article-title":"Osteoid osteoma mimicking refractory juvenile arthritis in a pediatric patient","volume":"51","author":"Baert","year":"2022","journal-title":"Scand J Rheumatol"},{"key":"B10","doi-asserted-by":"publisher","first-page":"63","DOI":"10.5114\/reum\/161185","article-title":"Overlapping clinical features of systemic juvenile idiopathic arthritis and SARS-CoV-2-related multisystem inflammatory syndrome in children","volume":"61","author":"Boyarchuk","year":"2023","journal-title":"Reumatologia"},{"key":"B11","doi-asserted-by":"publisher","first-page":"e15416","DOI":"10.1111\/1756-185X.15416","article-title":"Case report of unusual manifestation of mevalonate kinase deficiency syndrome mimicking juvenile idiopathic arthritis with systemic onset","volume":"27","author":"Bregel","year":"2024","journal-title":"Int J Rheum Dis"},{"key":"B12","doi-asserted-by":"publisher","first-page":"233","DOI":"10.1186\/s12879-025-10631-0","article-title":"Brucella bloodstream infection mimicking systemic juvenile idiopathic arthritis: a pediatric case report","volume":"25","author":"Ling","year":"2025","journal-title":"BMC Infect Dis"},{"key":"B13","doi-asserted-by":"publisher","first-page":"1748","DOI":"10.1136\/ard-2024-225853","article-title":"Systemic juvenile idiopathic arthritis and adult-onset still\u2019s disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR\/PReS recommendations for the diagnosis and management of still\u2019s disease","volume":"83","author":"De Matteis","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"B14","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1093\/mr\/roab115","article-title":"Performance of recent PRINTO criteria versus current ILAR criteria for systemic juvenile idiopathic arthritis: a single-centre experience","volume":"33","author":"Koker","year":"2023","journal-title":"Mod Rheumatol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"5739","DOI":"10.2147\/JIR.S343261","article-title":"Improving diagnosis and clinical management of acquired systemic autoinflammatory diseases","volume":"15","author":"Al-Hakim","year":"2022","journal-title":"J Inflamm Res"},{"key":"B16","doi-asserted-by":"publisher","first-page":"173","DOI":"10.1016\/j.amjmed.2021.07.038","article-title":"Fever of unknown origin (FUO)\u2014a call for new research standards and updated clinical management","volume":"135","author":"Wright","year":"2022","journal-title":"Am J Med"},{"key":"B17","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1186\/s12969-022-00700-y","article-title":"Monogenic disorders as mimics of juvenile idiopathic arthritis","volume":"20","author":"Furness","year":"2022","journal-title":"Pediatr Rheumatol Online J"},{"key":"B18","doi-asserted-by":"publisher","first-page":"2949","DOI":"10.1007\/s00296-024-05711-4","article-title":"Clinical outcomes of tocilizumab therapy in polyarticular and systemic juvenile idiopathic arthritis: a single-center analysis (2018\u20132022)","volume":"44","author":"Gazda","year":"2024","journal-title":"Rheumatol Int"},{"key":"B19","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1186\/s12969-018-0224-2","article-title":"Practice and consensus-based strategies in diagnosing and managing systemic juvenile idiopathic arthritis in Germany","volume":"16","author":"Hinze","year":"2018","journal-title":"Pediatr Rheumatol Online J"},{"key":"B20","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1186\/s12969-023-00944-2","article-title":"A rare case of coronary artery complication in a child with systemic juvenile idiopathic arthritis and macrophage activation syndrome: case report and literature review","volume":"22","author":"Zhang","year":"2024","journal-title":"Pediatr Rheumatol Online J"}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1885428\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T05:25:55Z","timestamp":1786944355000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1885428\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,8,17]]},"references-count":20,"alternative-id":["10.3389\/fped.2026.1885428"],"URL":"https:\/\/doi.org\/10.3389\/fped.2026.1885428","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,8,17]]},"article-number":"1885428"}}