A PILOT-STUDY OF INTERMITTENT INTRAVENOUS CYCLOPHOSPHAMIDE FOR THE TREATMENT OF SYSTEMIC-SCLEROSIS ASSOCIATED LUNG-DISEASE

Objective, Pulmonary fibrosis, a frequent manifestation of systemic sc lerosis (SSc), is considered incurable. Our aim was to assess the effe ct of therapy with intravenous (iv) cyclophosphamide on the course of pulmonary fibrosis in patients with SSc. Methods, Five patients with S Sc and clinical, laboratory, or radiographic findings of interstitial lung disease were treated with cyclophosphamide (1 g) administered iv monthly for 48 weeks. The dyspnea score, pulmonary function tests, art erial blood oxygen content, radiologic abnormalities, and bronchoalveo lar lavage (BAL) fluid cellularity were determined before and after th erapy. Results. The dyspnea score decreased by 42% after 48 weeks of t herapy. Forced vital capacity (FVC, percentage of predicted) increased by 7%, and carbon monoxide diffusing capacity (DLCO) decreased by 12% , but these changes were not statistically significant. Arterial blood oxygenation remained unchanged. At baseline, high resolution computed tomography of the lungs showed honey combing, reticulonodular, or gro und glass patterns in each patient examined. These radiologic abnormal ities did not improve during treatment. A marked decrease in BAL fluid cell number, but not in the percentage of neutrophils, was observed a fter therapy. Nausea and leukopenia were frequent but mild side effect s. One patient developed hemorrhagic cystitis. Conclusion. The results suggest that intermittent treatment with iv cyclophosphamide reduces the severity of dyspnea, but fails to improve FVC or DLCO, or cause re solution of radiologic abnormalities, in patients with SSc.