Review for best practice in clinical rheumatology juvenile systemic sclerosis - Updates and practice points

Abstract

Juvenile systemic sclerosis (jSSc) is a rare, severe autoimmune disease associated with life-threatening multiorgan inflammatory-driven fibrosis. Recognition early in the disease process, when treatment is more effective, is critical. We outline insights from the authors, who specialize and host jSSc cohorts, combined with recent literature review combining available juvenile-onset and applicable adult-onset studies regarding SSc evaluation, which can be extrapolated to children. Practice tips are provided for each main organ system.

Keywords: Clinical practice; Juvenile systemic sclerosis; Outcome measures and evaluation; Pediatric rheumatology; Scleroderma; Systemic sclerosis.