Background Currently data regarding long-term outcome of juvenile systemic sclerosis (jSSc) is scarce.
Objectives To describe the differences between patients with jSSc versus adult onset evaluated at a single medical center.
Methods Patients with disease onset before the age of 17 years were included in the jSSc group, while subjects with SSc onset after age 18 formed the adult-onset cohort. The 2 groups were compared with respect to disease classification, clinical, serological data, and mortality.
Results Seventeen jSSc cases seen between 1986 and 2011 were compared with 120 adult onset SSc cases. Mean age at onset in the jSSc group was 11.7±3.9 years, and the mean disease duration at the last follow-up was 19.3±7.8 years. The sex distribution was 7.5:1 (female:male). Among juvenile cases, 58.8% had diffuse SSc, while 41.2% had limited SSc. The clinical features seen in juvenile patients were: articular manifestations (arthralgia, arthritis, contractures and weakness) in the 100% of cases, Raynaud’s phenomenon in 94%, digital ulcers and telangiectasia in 53%, interstitial lung disease in 11.8%. Antinuclear antibody was positive in 100%, and anti-U1RNP in 64.7%. A global mortality of 17.6% was seen. Articular manifestations, anti-DNA and anti-U1RNP were seen more frequently in the jSSc group. Adult onset SSc develop a higher frequency of sclerodactyly and interstitial lung disease.
Conclusions Diffuse SSc, limited SSc, calcinosis, digital ulcers, pulmonary hypertension, anti-centromere, anti-Scl-70 and mortality shows a similar distribution between the 2 groups. Patients with jSSc presented a lower frequency of interstitial lung disease and sclerodactyly, but a higher expression of anti-DNA and anti-U1RNP.
Disclosure of Interest None Declared
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