Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia.

The present study compared the efficacy of cyclophosphamide combined with low-dose prednisolone in the treatment of idiopathic pulmonary fibrosis (IPF) with efficacy in idiopathic fibrosing nonspecific interstitial pneumonia fibrosing (NSIP). A total of 27 patients with IPF and 12 patients with fibrosing NSIP were included in this study. All patients had undergone surgical lung biopsy. The diagnoses were made based on clinical, radiological and pathological findings. All patients were treated with intermittent pulse therapy with methylprednisolone for 4 weeks, followed by cyclophosphamide with low-dose prednisolone. According to pulmonary function tests, four of 27 patients with IPF had improved, 22 remained unchanged, and one had worsened at the completion of pulse therapy. After 1 yr of combination therapy, four of 27 patients had improved, 14 remained unchanged, and nine had worsened. After pulse therapy, four of 12 patients with fibrosing NSIP had improved, and eight remained unchanged. After 1 yr of combination therapy, eight of 12 patients had improved, four remained unchanged, and none had worsened. Median survival of IPF patients was 4.1 yrs, which is significantly worse than that of fibrosing NSIP patients. In conclusion, patients with fibrosing nonspecific interstitial pneumonia had a more favourable response to combination therapy and a better survival than those with idiopathic pulmonary fibrosis.