Progressive pulmonary fibrosis complicating cyclophosphamide therapy

A 26-year-old woman with extra-pulmonary Wegener's granulomatosis was treated with cyclophosphamide for 3.25 years, cumulated dose of 91 g. Six months before cessation of therapy discrete radiological signs of apical fibrosis appeared. The changes were progressive regardless of discontinuation of cyclophosphamide and led to severe restrictive ventilatory defect.