New insights into the pathogenesis and management of juvenile systemic sclerosis

2015 Systemic sclerosis remains one of the most clinically challenging diseases for rheumatologists and patients continue to suffer from a considerable degree of morbidity and mortality. Even though the pathogenesis of the disease remains complex and poorly understood, new research over the past several years have brought us closer to an understanding of the underlying disease mechanisms and complications (e.g., fibrosis, immunological abnormalities, vasculopathy). Emerging clinical data suggest that juvenile Systemic Sclerosis (jSSc) differs from systemic sclerosis in adults in both its presentation and clinical course. This review summarizes recent developments in the understanding of the immune pathogenesis and new therapeutic options for the treatment of jSSc.