Systemic sclerosis (formerly scleroderma) is a relatively rare autoimmune connective tissue disease which symmetrically affects the skin and affects the internal organs. There are two types: limited cutaneous and diffuse cutaneous. Each type is categorized by different clinical, systemic, and serologic findings. Autoantibodies can be used to predict phenotype and internal organ involvement. Systemic sclerosis can affect the lungs, gastrointestinal system, kidneys, and heart. Pulmonary and cardiac disease are the leading causes of death, so screening for these conditions is important. Early management of systemic sclerosis is paramount to prevent progression. Numerous therapeutic interventions for systemic sclerosis exist, but there is no cure. The goal of therapy is to improve quality of life by minimizing specific organ-threatening involvement and life-threatening disease.
Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.