Pulmonary fibrosis occurs most patients who have scleroderma. It progresses to severe restrictive lung disease in about 15% of patients and remains a major cause of death in this disease. Risks for developing pulmonary fibrosis include diffuse cutaneous scleroderma and anti-Scl-70 antibodies, and risks for developing progressive pulmonary fibrosis and death include low pulmonary function test results at presentation and lung inflammation. Early evaluation of the extent and severity of pulmonary fibrosis and the presence of lung inflammation is key, so that therapy can be given to patients who are at higher risk of progressive pulmonary fibrosis before they develop severe functional impairment occurs. Evaluation often includes pulmonary function tests, HRCT of the lungs, Doppler echocardiogram, and bronchoalveolar lavage. For patients who are unwilling or unable to participate in therapeutic trials that target pulmonary fibrosis in scleroderma, therapy with oral or intravenous cyclophosphamide is often given.