May 19 2012 Prednisone, Azathioprine, and N-Acetylcysteine for Pulmonary Fibrosis

The Idiopathic Pulmonary Fibrosis Clinical Research Network

N Engl J Med 2012;  366:1968-1977May 24, 2012DOI:  10.1056/NEJMoa1113354

Idiopathic pulmonary fibrosis is a chronic, progressive lung disease of unknown cause characterized by the histopathological pattern of usual interstitial pneumonia.1 The median survival of patients with idiopathic pulmonary fibrosis after diagnosis is 2 to 5 years.1

The use of glucocorticoids or immunosuppressive agents has been the conventional approach to the treatment of patients with this disease. A consensus-based guideline suggested that a two-drug regimen (a combination of prednisone and either azathioprine or cyclophosphamide) be used in a subgroup of patients with idiopathic pulmonary fibrosis.2 A survey of pulmonologists showed that almost 50% used a regimen of either two drugs (azathioprine plus prednisone) or three drugs (azathioprine, prednisone, and N-acetylcysteine [NAC]) when therapy was offered to their patients with mild idiopathic pulmonary fibrosis.3


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