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Introduction Juvenile systemic sclerosis (jSSc) is a rare disease with a prevalence of 3 in 1 000 000 children. In adult patients antic-scl 70 antibodies correlate with the diffuse subtype and a specific organ involvement pattern. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects multinational data including clinical presentation, antibody profile and patient and physician reported outcomes.Material and Methods The jSScC enrolls jSSc patients who developed the first non-Raynauds symptom before the age of 16 years and are under the age of 18 years at the time of inclusion. We extracted the data at the time of inclusion in the registry from patients in the jSScC, including clinical manifestations and patient and physician reported outcomes until 15th of December 2024. Unknown antibody status for anti-Scl70 antibodies was an exclusion criteria.Results 260 patients from the cohort. 31% (n=80) were anti Scl-70 positive (antiScl70+). the anti-Scl70+ and anti-Scl70-group were similar regarding disease duration at time of inclusion (approximately 2.5 years), mean age at first non-Raynauds involvement (11 years of age), ethnicity, subtype of SSc and positive for anti-centromere antibodies. C-reactive protein (CRP) was elevated in 23% in the anti-Scl70+ group compared to 5% in the anti-Scl70- group (p <0.001). Anti-Scl70+ patients had less overlap features (18% vs 30%, p<0.001). Patients with anti-Scl70+ had higher rate of nailfold capillary changes (84% vs 74%, p=0.015), history of ulcerations (61% vs 47%, p=0.345) and telangiectasia (29% vs 40%, p=0.049). There were no differences regarding number of patients with decrease FVC<80%, DLCO<80% or positive HRCT findings for interstitial lung disease. Rate of pulmonary hypertension was 8% in anti-Scl70+ compared to 5% in anti-Scl70-. No differences in cardiac, gastrointestinal or renal involvement were found. No renal crisis occurred. Patient rated the global disease damage significantly higher in the anti-Scl-70+ group (48 vs 30 (VAS 0-100), p=0.031) and the Raynaud activity (45 vs 20 (VAS 0-100), p=0.022).Conclusions Anti-Scl70+ patients have distinct features compared to anti-Scl- patients, but interstitial lung disease is not more common at inclusion in the registry, despite the higher rate of CRP elevation. Pediatric organ involvement pattern clearly differs from adults.Abstract P.333 Table 1Comparison anti-Scl70 positive and negative patients at time of inclusion into the cohort