Journal
RHEUMATIC DISEASE CLINICS OF NORTH AMERICA
Volume 29, Issue 2, Pages 371-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/S0889-857X(03)00025-5
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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.
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