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Immunosuppressant Therapy for Idiopathic Pulmonary Fibrosis

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Summary

Idiopathic pulmonary fibrosis (IPF) is associated with progressive respiratory failure and is usually fatal. Corticosteroid therapy is effective in less than half of the patients in whom it is tried.

Recently, it has become apparent that immunosuppressant drugs, especially azathioprine and cyclophosphamide, have a role in the management of IPF patients. We review the indications, previous experience, proposed mechanism of action and apparent effectiveness of these drugs in patients with IPF. Both drugs appear to be more effective than corticosteroids alone in selected patients with IPF. Although not all patients respond to either therapy, the drugs seem to be indicated in patients with mild to moderate symptomatic disease. Factors associated with a better prognosis and response to therapy in pulmonary fibrosis include patients being less than 50 years old, having vital capacities > 50% of predicted, and the presence of an underlying collagen vascular disease. If monitored appropriately, the toxicity of these agents is minimal in most patients.

With the limited information now available, use of immunosuppressive agents should be considered in all patients with pulmonary fibrosis, especially those failing to respond to corticosteroid therapy.

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Correspondence to Dr Robert P. Baughman.

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Baughman, R.P., Lower, E.E. Immunosuppressant Therapy for Idiopathic Pulmonary Fibrosis. Clin. Immunother. 6, 431–442 (1996). https://doi.org/10.1007/BF03259365

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Keywords

  • Cyclophosphamide
  • Idiopathic Pulmonary Fibrosis
  • Pulmonary Fibrosis
  • Lupus Nephritis
  • Interstitial Lung Disease