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Systemic Sclerosis: Evaluation and Treatment

Winfred Frazier, MD, MPH
Courtney Goettel, MD, MS
Parul Chaudhri, DO

American Family Physician. 2026;113(4):349-357.

Author disclosure: No relevant financial relationships.

This clinical content conforms to AAFP criteria for CME.

Systemic sclerosis is a rare autoimmune connective tissue disease characterized by progressive fibrosis of the skin and internal organs, vasculopathy, and the presence of specific autoantibodies. Despite its low prevalence, systemic sclerosis is associated with high morbidity. Early features often include Raynaud phenomenon, hand edema, and fatigue. Diagnosis requires a comprehensive approach, including clinical assessment, laboratory evaluation, imaging, and pulmonary function testing. The American College of Rheumatology and European Alliance of Associations for Rheumatology (formerly the European League Against Rheumatism) provide classification criteria and updated treatment recommendations. Management focuses on addressing eight disease domains: Raynaud phenomenon, digital ulcers, pulmonary artery hypertension, interstitial lung disease, renal crisis, gastrointestinal involvement, skin fibrosis, and musculoskeletal involvement. Vasodilator therapy is first-line treatment for Raynaud phenomenon, whereas phosphodiesterase-5 inhibitors and intravenous iloprost are used to treat digital ulcers. Combination therapy with phosphodiesterase-5 inhibitors and endothelin receptor antagonists is first-line treatment for pulmonary artery hypertension. Mycophenolate mofetil is the preferred treatment for interstitial lung disease.

WINFRED FRAZIER, MD, MPH, FAAFP, is an associate professor and vice chair for education in the Department of Family Medicine at the University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.

COURTNEY GOETTEL, MD, MS, FAAFP, is assistant director of the Williamsport Family Medicine Residency Program at the University of Pittsburgh Medical Center, Williamsport, Pennsylvania.

PARUL CHAUDHRI, DO, FAAFP, is associate program director, director of osteopathic education, and an assistant professor of family medicine at the University of Toledo Family Medicine Residency Program, Toledo, Ohio.

Address correspondence to Winfred Frazier, MD, MPH, FAAFP, at frazierw@pitt.edu.

Author disclosure: No relevant financial relationships.

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