Cyclophosphamide for scleroderma interstitial lung disease. Tashkin DP, Elashoff R, Clements PJ et al.: Cyclophosphamide versus placebo in scleroderma lung disease. N. Engl. J. Med. (2006) 354(25):2655-2666

Abstract

Systemic sclerosis (scleroderma) is a chronic debilitating disease that is caused by the occurrence of fibrotic changes and vascular abnormalities at various levels such as: skin, lungs, kidneys or heart. Lung involvement in scleroderma is represented by scleroderma interstitial lung disease and by pulmonary arterial hypertension, and is one of the leading causes of mortality in this disease. Pulmonary arterial hypertension can be successfully treated with specific therapies such as sildenafil, bosentan or epoprostenol, whereas only cyclophosphamide has been shown to be effective for interstitial lung disease. The discussed study has shown that cyclophosphamide improved lung function, functional status and health-related quality of life, and reduced dyspnea in scleroderma patients. Most of these patients had acute alveolitis although higher incidences of treatment-related leukopenia and neutropenia were also detected.