Immunosuppressive therapy of idiopathic pulmonary fibrosis with cyclophosphamide

Idiopathic pulmonary fibrosis is an interstitial lung disease with poor prognosis. First line therapy consists mainly of corticosteroids. In case of failure azathioprine and cyclophosphamide are used, but efficacy of immunosuppressive treatment is limited. We retrospectively analyzed 18 IPF- patients resistant to corticosteroid-therapy, who have been treated with intermittent cyclophosphamide once monthly. In 11 of 17 evaluable patients the progressive decline of respiratory function could be stopped, 6 even showed significant improvement of parameters. Response to therapy was favorable, if vital capacity was better than 60% predicted and if BAL showed lymphocytosis. In patients with more impaired vital capacity and neutrophilia in BAL cyclophosphamide was less effective. A higher incidence of respiratory tract infections was observed in some patients, therefore close follow-up and a well balanced use of antibiotics is recommended.