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Introduction Juvenile systemic sclerosis(jSSc) is an orphan disease with a prevalence of 3 in 1.000.000 children. The Juvenile Systemic Scleroderma Inception cohort(jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects data prospectively in jSSc, allowing the evaluation of the development of organ involvement and patient and physician reported outcomes in jSSc over time.Material and Methods The jSScC cohort enroll 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 reviewed jSScC patient clinical data and patient and physician reported outcomes, who had 12 months follow up from the time of inclusion until 15th of December 2024.Results There were 150 patients with 12 months follow up included in the jSScC (79% Caucasian, 78% female). Diffuse subset occurred in 75% of the patients. Mean disease duration at the time of inclusion in the cohort was 2.5 years. Median age of onset of Raynauds was 10 years old (YO) and median age of first non-Raynauds organ involvement was 10.6 YO. 92% of the patients were treated with DMARDs at time point 0 and 97% at time point 12 months. There was a significant improvement in the mean modified Rodnan skin score (MRSS) in 12 months (12 vs 8; p=0.031). The MRSS decreased 10% or more in 47% and increased 10% or more in 25% of the patients. After 12 months 7 patients decreased FVC to <80% and 4 patients decreased DLCO to <80%, but 6 patients improved FVC or DLCO to >80%, in the mean of the group no significant change. The number of patients with a 6 Minute Walk Test below the 10th percentile for age decreased from 67% to 45% (p=0.058). The number of patients with swollen joints decreased significantly from 16 to 7% (p=0.019). No changes were seen in cardiac, renal or gastrointestinal involvement. No renal crisis occurred. All assessed patient and 2 of the 3 physician reported outcomes improved significantly.Conclusions Skin and musculoskeletal clinical features improved over 12 months, with almost all patients on DMARDs, supporting the response of these features to therapy. It was promising that internal organ involvement, like cardiac, lung and gastrointestinal, did not significantly worsen after 12 months. The most striking observation is the improvement of all assessed patient and physician reported outcome measures over 12 months in this largest international cohort of jSSc patients.Abstract P.334 Table 1Evolvement of organ involvement and physician and patient reported outcomes