Systemic Sclerosis Associated Interstitial Lung Disease

Systemic sclerosis is a disease of typically middle aged women, with significant morbidity and mortality predominantly caused by lung involvement. Screening for and diagnosis of interstitial lung disease is critical given the decreased survival associated with progressive fibrotic lung disease. Monitoring of disease is necessary once the diagnosis has been made, and surgical lung biopsy is usually unnecessary. Treatment centers on immunosuppressants without additional steroids with attention to identification and management of co-existing complications, such as pulmonary hypertension and gastro-esophageal reflux, as well as supportive therapy with supplemental oxygen and exercise. Referral for lung transplant evaluation should be considered when there is persistent progression.