Survival in idiopathic pulmonary fibrosis-cytotoxic agents compared to corticosteroids

Respir Med. 2006 Feb;100(2):340-7. doi: 10.1016/j.rmed.2005.05.008. Epub 2005 Jul 5.


Study objective: To compare the survival of patients with IPF treated retrospectively with corticosteroids alone, to survival of patients treated with immunosuppressive and corticosteroids combined.

Design: Non-randomized retrospective cohort study.

Setting: Three tertiary centers in Brazil.

Patients: Eighty-two IPF patients were included. The diagnosis was confirmed by open lung biopsy in 48. Patients received either corticosteroids alone (group I) or cytotoxic agents in addition to corticosteroids (group II).

Measurements and results: The primary end-point was mortality. Secondary outcome included longitudinal changes in FVC. Mean age was 66+/-8 years. FVC was 71+/-17% of predicted. There were 48 deaths during the study period (59%), 44 secondary to respiratory causes. From preliminary univariate analysis, for the group as a whole, worse survival was found to be associated with FVC% <70% of predicted (P=0.004), evidence of disease progression by follow-up FVC measurements (P=0.01), and pharmacologic treatment (P=0.014). Median survival was 25 months for the group I, and 45 months for the group II (Log-Rank=6.45, P=0.01). After adjusting for FVC > or =70% and<70% of predicted, there was evidence to indicate that survival was associated with recommended pharmacologic treatment only in patients with FVC> or =70% (Log Rank=6.84, P=0.009).

Conclusions: The combination of immunosuppressive agents and prednisone results in better survival when compared to prednisone alone in patients with IPF. The benefit seems to occur only in patients with less severe disease, as reflected by FVC> or =70%.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Aged
  • Antimetabolites, Antineoplastic / therapeutic use*
  • Azathioprine
  • Brazil / epidemiology
  • Cohort Studies
  • Cyclophosphamide / therapeutic use*
  • Female
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Male
  • Methylprednisolone / therapeutic use*
  • Pulmonary Fibrosis / drug therapy*
  • Pulmonary Fibrosis / physiopathology
  • Retrospective Studies
  • Survival Analysis
  • Treatment Outcome
  • Vital Capacity / drug effects
  • Vital Capacity / physiology


  • Antimetabolites, Antineoplastic
  • Glucocorticoids
  • Immunosuppressive Agents
  • Cyclophosphamide
  • Azathioprine
  • Methylprednisolone