Systemic Sclerosis: A Comprehensive Approach to Diagnosis and Management

Systemic sclerosis is a progressive multisystem disease characterized by autoimmunity, endothelial cell dysfunction, and excessive fibrosis in the skin and internal organs. Systemic sclerosis is divided into limited and diffuse cutaneous disease based on the presence of skin fibrosis distal to the elbows and knees (limited) or proximal to the elbows and knees (diffuse). Pharmacologic treatment is directed at the underlying manifestations of the disease (eg, mycophenolate mofetil for diffuse skin thickening and symptomatic progressive interstitial lung disease, proton pump inhibitors for gastroesophageal reflux disease, and calcium channel blockers for Raynaud phenomenon).