Management of systemic sclerosis: A dermatologist's approach

Abstract

Systemic sclerosis (SSc) is an autoimmune connective tissue disease affecting the blood vessels, skin, and internal organs. The main pathogenic mechanisms are vascular dysregulation, tissue fibrosis due to collagen and extracellular protein deposition, and autoantibody production due to immune dysfunction. The treatment also targets these mechanisms. Various criteria including the American College of Rheumatology and the European League against Rheumatism are laid down for the clinical diagnosis of scleroderma, and treatment is tailored to the systems involved. In the initial stages of the SSc, immunosuppressants are more useful, whereas in the later stages, antifibrotic therapies are important. Vasospasm and endothelial dysfunction are seen in all stages of the disease, and hence, vascular therapies are needed throughout the course of the disease. Special care should be taken to rule out gastrointestinal, pulmonary, and renal system involvement if the primary treatment is by a dermatologist.