Clinical Features of Systemic Sclerosis

Hospital Physician January 2008 33 S ystemic sclerosis, or scleroderma, is a rare connective tissue disease of unknown etiology. The word scleroderma, derived from the Greek words scleros for hard and derma for skin, refers to the hallmark finding of chronic hardening and thickening of the skin that occurs in this disease. The clinical manifestations of systemic sclerosis range from localized skin lesions affecting small areas (localized scleroderma) to systemic involvement in which multiple organ systems are affected. Systemic sclerosis is characterized by inflammation and fibrosis leading to changes in the blood vessels, skin, synovium, skeletal muscle, and internal organs. Morbidity and mortality in systemic sclerosis depend on the organ or system involved. Early recognition and diagnosis are important because effective therapies for many of the systemic manifestations are available, but timely treatment is required. This article reviews the clinical features of systemic sclerosis and provides a limited discussion on therapeutic options.