Juvenile systemic sclerosis: review of 15 patients

Results Results: Fifteen patients were identified and included in the analysis, 3 of them were overlap syndromes. Eleven girls (73%), 13 (87%) Caucasians, with a mean age at diagnosis of 11.1±3.0 (3–15) years and a mean disease duration of 7.2±4.2 years (8 months-17 years). In 14 (93%) cases, the first symptom attributable to JSSc was Raynaud’s phenomenon, followed by arthritis and/or puffy hands (9 patients, 60%). At disease diagnosis 12 (80%) patients presented periungual capillaropathy and in 8 patients, pulmonary involvement was documented, despite the absence of respiratory complaints. Cumulative disease manifestations as well as complications developed during follow-up are shown in table 1. All but one child were ANA positive (93%), 7 tested positive for anti-Scl70, 2 positive for anti-RNP and 1 for anti-fibrillarin antibodies. There were no cases of anticentromere antibodies. Immunosuppressants (93%), proton pump inhibitors (80%), calcium channel blockers (53%) and corticosteroids (60%) were the most common therapeutic options. Five and four children were treated with prostacyclin analogues and ET-1 receptor antagonist, respectively. One child needed autologous bone marrow transplant due to severe refractory disease. An improvement of skin thickening and stabilization of pulmonary involvement was documented in most cases. No deaths were registered in this cohort. Table 1.