Juvenile systemic sclerosis: a follow-up study of eight patients.

Juvenile systemic sclerosis (jSSc) is a rare form of systemic sclerosis (also known as scleroderma). Fewer than 10% of SSc cases have their onset before age 20 and fewer than 2% before the age of 10. Few case reports and cohort studies on jSSc have been published. Our objective was to assess the clinical and laboratory characteristics of eight cases of juvenile-onset SSc followed up at our institution. Clinical manifestations of SSc were recorded, and immunologic laboratory tests including antinuclear antibodies (ANAs), anti-centromere antibodies (ACAs), and anti-Scl-70 antibodies were assessed. The female-to-male ratio was 7:1, and age at onset ranged from 3-17 years. At the time of analysis, 2 of 8 patients were still under 18 years of age, and 6 of 8 patients were adults. The mean follow-up period from the onset of disease was 19.1 years. Raynaud's phenomenon was present in all cases. Only 2 of 8 patients had diffuse SSc. During follow-up, pulmonary fibrosis developed in only 2 of 8 patients and cardiovascular manifestations in 3 of 8 patients. Secondary sicca syndrome was present in 2 of 8 cases. Regarding immunologic laboratory markers, 7 of 8 patients were ANA positive. However, none of these patients ever carried anti-Scl-70 antibodies, and only 2 of 8 patients had ever had ACA seropositivity. Our results suggest that in jSSc, Raynaud's phenomenon is more severe, whereas internal organ manifestations and the frequency of autoantibodies are far less pronounced than in adult-onset SSc. Also, the survival rate and final outcome of patients with jSSc appear to be better than those in patients with adult-onset SSc.