Early Stage of Fulminant Idiopathic Pulmonary Fibrosis Cured by Intense Combination Therapy Using Cyclophosphamide, Vincristine, and Prednisone

Abstract

A 19-year-old patient was treated who developed a life-threatening pulmonary insufficiency after having suffered from a flu-like illness for 13 days. Lung biopsy revealed an early stage of idiopathic pulmonary fibrosis. Chemotherapy by prednisone alone proved ineffective. A combination of prednisone and massive doses of cyclophosphamide and vincristine, however, was effective in halting the rapid progression of the disease. The patient recovered gradually; the results of the pulmonary function tests were close to normal 2 weeks after the onset of therapy. Lung biopsy showed normal lung histology 7 months later. The patient has had no complaints at all for more than 3 years after the episode so that we may assume that he recovered fully.