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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 anticentromere antibodies correlate with the limited subtype. The Juvenile Systemic Scleroderma Inception cohort (jSScC) is the largest cohort of jSSc patients in the world. The jSScC collects data multinational, including clinical presentation, auto-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 reviewed, data of patients in jSScC at the time of inclusion in the registry, including clinical data and patient and physician reported outcomes until 15th of December 2024. Absence of results of anticentromere antibodies was an exclusion criterion.Results We analyzed 197 patients. 78% female, 66% Caucasian, 11 (6%) were anticentromere antibody positive (AC+). Diffuse subtype was present in 45% of the AC+ and in 68% of the AC- patients. There were no differences regarding median disease duration (2.5-2,8 years 9 at registry entrance or ethnicity between the two groups. The onset of Raynauds was significantly earlier in the AC+ group (7.2 vs 10.1 years, p=0.042). Moreover, the median age of the first non-Raynaud organ involvement was significantly earlier in the AC+ (6.8 to 10.4 years, p=0.039). Anti-scl 70 positivity was not significantly different (18% in AC+ vs 29% in AC-). The AC+ group had more lung involvement. Decreased FVC <80% was seen in 63% of AC+ (5/8), compared to 27% of AC- patients (39/144) (p=0.032). DLCO < 80% occurred in 86% of the AC+ patients (6/7) and 29% of AC- (45/115) (p=0.027). Lung HRCT findings occurred in 88% of the AC+ (7/8) and in 43% of AC-patients (65/152) (p=0.015). The AC+ patients had significantly more decreased Body Mass Index of < - 2 z score and calcinosis (p=0.044 and p=0.045, respectively). The history of ulceration was more frequent in the AC- group. The patient ratedRaynaud activity in the last 7 days was significantly higher in the AC+ group.Conclusions jSSc patients with AC+ show different characteristics in the largest cohort of jSSc patients. The number of AC+ patients is significantly lower than in an adult systemic sclerosis population. Interestingly, the AC+ patients have a significantly lower age at onset of disease and higher pulmonary involvement. We need further data to reconfirm this unique observation.Abstract P.332 Table 1Differences in clinical presentation in anti-centromere positive versus negative patients at time of inclusion in the cohort