Background Juvenile scleroderma (JS) is a rarely seen chronic connective tissue disorder. According to organ involvement, the disease is divided into two main forms: systemic and localized. Localized scleroderma (JLS) is characterized with sclerosis of the skin but internal organs involvement is not expected. Juvenile systemic sclerosis (JSS) is characterized by both cutaneous and internal organ involvement. Clinical features are insidious and it can take years until complete clinical picture develops.
Objectives The aim of the study was to investigate main demographic and clinical characteristics of patients with JS, followed up at our department. Additionally, we aim to share our experience of this rare condition.
Methods Patients with JS were included in cross-sectional study. Demographic data were taken from disease history of patients. Clinical features, laboratory results and treatment options were evaluated at last clinical visit.
Results A totally of 46 patients were included in the study: 40 (86,9%) female, 6 (13%) male. The mean age of patients was 14±3 years, mean age at disease onset was 9,1±4,23 years and the mean age at diagnosis was 10,2±3,4 years. Twenty six patients (56,5%) were diagnosed with JSS and 20 (43,4%) had JLS. Skin involvement and peripheral vasculopathy were the most common clinical features. Musculoskeletal involvement takes a second place. Gastro-intestinal (GIS) involvement was present only in systemic sclerosis group. Six patients (13%) had lung fibrosis. Pulmonary hypertension was found in 6,5% of all JS patients but its percentage was higher among patients with JSS (11,5%). None of patients had neurological and renal involvement. All JSS patients were positive while 40% of JLS patients were negative for ANA. Anti Scl 70 antibody was positive only among patients with JSS. Combination of MTX+corticosteroids was used in 15 patients (32,6%). Vasoactive agent was added in therapy of 20 patients (43,5%) which all belong to JSS group. While 31 patients were in remission (67,4%), 15 (32,6%) had active disease in last visit. All patients with active disease belonged to systemic group.
Conclusions Juvenile scleroderma is rarely seen multisystemic disease. Unlike adults, cardiovascular and pulmonary involvement is uncommon among children. Early diagnosis, regular follow up and appropriate treatment are of high importance in clinical course and disease prognosis.
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Disclosure of Interest None declared