POS0172 DIFFUSE JUVENILE SYSTEMIC SCLEROSIS PATIENTS SHOW DISTINCT ORGAN INVOLVEMENT AND HAVE MORE SEVERE DISEASE IN THE LARGEST jSSc COHORT OF THE WORLD. RESULTS FROM THE THE JUVENILE SCLERODERMA INCEPTION COHORT. WWW.JUVENILE-SCLERODERMA.COM

Abstract

Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children. In adult patients there are significant differences between the clinical presentation of diffuse and limited subtypes. We reviewed clinical differences in presentation of subtypes in patients in the juvenile systemic scleroderma inception cohort up to 2021. Method(s): We reviewed the clinical baseline characteristics of the patients, who were recruited to the juvenile scleroderma inception cohort (jSScC) till 1st of December 2021. jSScC is a prospective cohort of 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. Result(s): 210 patients with jSSc were included in the cohort, 71% (n=162) had diffuse subtype. The median age at onset of Raynaud phenomenon was 10.4 years (7.3-12.9) and the median age at the first non-Raynaud symptom was 10.9 years (7.4-13.2). Median disease duration was 2.5 years (1-4.4) at the time of inclusion. The female/male ratio was significantly lower in the djSSc subtype (3.7:1 versus 5:1, p< 0.001). Antibody profile was quite similar, with the exception of a significantly higher number of anticentromere positive patients in the ljSSc (12% versus 2%, p=0.013). Decreased FVC < 80% was found in approximately 30% and decreased DLCO < 80% was found in around 40% in both subtypes. Pulmonary hypertension assessed by ultrasound was identified in 5% in both groups. Patients with diffuse subtype had significantly higher modified Rodnan Skin Score (mRSS) (16 versus 4.5, p< 0.001), sclerodactyly (84% versus 60%, p< 0.001), history of digital ulceration (62% versus 31%, p< 0.001), decreased Body Mass Index (BMI) < 0.001) and physician reported global disease damage (VAS 0-100) (30 versus 15, p=0.014) was significantly higher in djSSc. Conclusion(s): In this jSSc cohort, the largest in the world, djSSc patients have a significantly more severe disease than ljSSc patients. Interestingly, we found no differences regarding interstitial lung disease and pulmonary hypertension. This project was supported by an unrestricted grant from .,Joachim Herz Stiftung".