Interstitial lung disease in scleroderma

Pulmonary fibrosis is a common complication of scleroderma that can cause severe restrictive lung disease and death in a subset of patients. The clinical management of patients who have scleroderma with pulmonary fibrosis remains a challenge. Rheumatologists must work with other members of the health care team to assess the patient's risk of developing progressive pulmonary fibrosis; make decisions about the timing, nature, and interpretation of diagnostic tests; and initiate and discontinue treatment. This article summarizes the prevalence, natural history, risk factors, pathology, mechanisms of tissue damage, and evaluation and therapy of pulmonary fibrosis in scleroderma.