Systemic sclerosis (systemic scleroderma)

Catherine H. Orteu, Christopher P. Denton


Systemic sclerosis is a multisystem autoimmune disease that causes fibrosis in the skin and internal organs with associated vascular and inflammatory manifestations including Raynaud phenomenon. It has high mortality due to cardiac, pulmonary and renal complications and substantial morbidity from pruritus, pain, digital ulceration, calcinosis, telangiectases and gastrointestinal tract and musculoskeletal involvement. Different subsets of the disease are recognized based on the extent of skin involvement, the presence of hallmark autoantibody reactivities, and more recently on gene expression signatures in the skin. Up to 20% of systemic sclerosis cases have features of overlap with another autoimmune rheumatic disease. Limited and diffuse subsets carry different risks of organ‐based complications. Early and systematic screening to identify the burden of disease and individualize risk is a cornerstone of modern management, which has led to the earlier institution of therapy and significant improvements in outcome over the past two decades.
Keywords systemic sclerosis, limited systemic sclerosis, diffuse systemic sclerosis, overlap connective tissue disease, sclerodactyly, Raynaud phenomenon, autoantibodies, modified Rodnan skin score, genetic susceptibility, scleroderma‐like disease


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