Systemic sclerosis in children and older juvenile-onset patients: real-life map by the Egyptian college of Rheumatology

Abstract

The aim of this work was to present the demographic and clinical features of children with juvenile systemic sclerosis (JSSc) compared to their older juvenile-onset (Jo-SSc) counterparts. The study included 12 JSSc children (< 16 years) and 54 Jo-SSc patients > 16 years (including adolescents and adults) recruited from 15 tertiary care centers across Egypt. Patients were classified as diffuse (dcSSc), limited cutaneous SSc (lcSSc) and overlap. The mean age at disease onset was 14 ± 2.5 years. Patients were 62 females and 4 males (F: M 15.5:1); 37 lcSSc, 26 dcSSc and 3 overlap. Disease features were generally comparable across five regions of Egypt. Pitting scars and hand puffiness were significantly more frequent in JSSc (58.3% and 41.7%) compared to Jo-SSc (16.7% and 14.8%) (p = 0.006 and p = 0.049, respectively) while pulmonary hypertension was more common in Jo-SSc (40.7% vs. 8.3%, p = 0.045). All males, currently with Jo-SSc, had gastrointestinal involvement versus n = 24 (38.7%) in females, p < 0.0001. To conclude, SSc in children and in those who grow into adulthood remains a rare disease. Pitting scars and hand puffiness were more frequent in children with JSSc while pulmonary hypertension became prominent as they grew. Children characteristics are special for each nation yet with similarities in some features.