{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T09:36:20Z","timestamp":1790847380730,"version":"4.1.0"},"reference-count":26,"publisher":"The Journal of Rheumatology","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"accepted":{"date-parts":[[2012,11,23]]},"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:sec><jats:title>Objective.<\/jats:title><jats:p>Systemic juvenile idiopathic arthritis (SJIA) frequently leads to disability and damage. Predictive factors for a poor outcome include persistent systemic features and younger age at onset. We describe and analyze disease features in patients with early-onset (EO) SJIA (disease onset before age 18 mo) and compare them to patients with later-onset (LO) disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods.<\/jats:title><jats:p>Clinical features at onset, activity measures (occurrence of macrophage activation syndrome, remission), and outcome measures for disability [Childhood Health Assessment Questionnaire (CHAQ) \u2265 0.5] and damage [radiographic joint destruction, Juvenile Arthritis Damage Index (JADI) score, growth retardation] observed during followup were analyzed retrospectively in patients with SJIA followed for \u2265 3 years since disease onset.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results.<\/jats:title><jats:p>In total 132 patients were included. SJIA started at age \u2264 18 months in 19 (14%) patients and at a later age in 113 (86%) children. At onset, serositis (p &lt; 0.01) and hepatomegaly (p &lt; 0.05) were more frequent in EO patients, who also exhibited lower hemoglobin levels (p &lt; 0.03) and higher platelet counts (p &lt; 0.03) than patients with LO. Macrophage activation syndrome occurred in 20 patients (11 EO and 9 LO; p &lt; 0.0001). Remission was achieved by 49 patients (37%; 4 EO and 45 LO). At last visit, destructive hip disease (p &lt; 0.04), growth retardation (p &lt; 0.01), radiographic damage (p &lt; 0.02), and disability (p &lt; 0.04) were more frequent in patients with EO disease, who had higher JADI scores (p &lt; 0.003).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion.<\/jats:title><jats:p>Patients with EO exhibited a more aggressive and destructive disease course than patients with LO SJIA.<\/jats:p><\/jats:sec>","DOI":"10.3899\/jrheum.120386","type":"journal-article","created":{"date-parts":[[2013,1,16]],"date-time":"2013-01-16T04:25:40Z","timestamp":1358310340000},"page":"329-334","source":"Crossref","is-referenced-by-count":50,"title":["Patients with Very Early-onset Systemic Juvenile Idiopathic Arthritis Exhibit More Inflammatory Features and a Worse Outcome"],"prefix":"10.3899","volume":"40","author":[{"given":"RICARDO A.G.","family":"RUSSO","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MAR\u00cdA M.","family":"KATSICAS","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2013,1,15]]},"reference":[{"key":"2025021211251273000_40.3.329.1","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1038\/ncprheum0084","article-title":"Systemic juvenile idiopathic arthritis: Diagnosis, management, and outcome","volume":"2","author":"Woo","year":"2006","journal-title":"Nat Rev Rheumatol"},{"key":"2025021211251273000_40.3.329.2","doi-asserted-by":"publisher","DOI":"10.1016\/S0950-3579(98)80018-6"},{"key":"2025021211251273000_40.3.329.3","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)80427-5"},{"key":"2025021211251273000_40.3.329.4","first-page":"211","article-title":"Systemic onset juvenile chronic arthritis, polyarticular pattern and hip involvement as markers for a bad prognosis","volume":"19","author":"Modesto","year":"2001","journal-title":"Clin Exp Rheumatol"},{"key":"2025021211251273000_40.3.329.5","doi-asserted-by":"publisher","DOI":"10.1002\/art.11300"},{"key":"2025021211251273000_40.3.329.6","doi-asserted-by":"crossref","unstructured":"Petty RE Cassidy JT . Systemic arthritis. In: Cassidy JT Petty RE Laxer RM Lindsley CB , editors. Textbook of pediatric rheumatology. 5th ed. 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