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
Keywords: Fibrosis, interstitial lung disease (ILD), review, scleroderma, severe.
Rights & PermissionsPrintExport