Treatment
As of 2006, there was no consensus on treatment nor satisfactory treatment.
A 2010 Cochrane analysis found that pirfenidone significantly improved progression-free survival. In 2011, pirfenidone was approved for use in Europe under the brand name Esbriet.
There is a lack of large, randomized placebo-controlled trials of therapy for IPF. Moreover, many of the earlier studies were based on the hypothesis that IPF is an inflammatory disorder, and hence studied anti-inflammatory agents such as corticosteroids. Another problem has been that studies conducted prior to the more recent classification of idiopathic interstitial pneumonias failed to distinguish IPF/UIP from NSIP in particular. Hence, many patients with arguably more steroid-responsive diseases were included in earlier studies, confounding the interpretation of their results.
A large randomized, controlled trial (PANTHER-IPF) found that the combination of prednisone, azathioprine, and N-acetylcysteine had a significantly higher death rate than placebo (8 vs. 1), and the trial was terminated.
Other treatments studied have included interferon gamma-1b, the antifibrotic agent pirfenidone, and bosentan. Bosentan are currently being studied in patients with IPF while interferon gamma-1b is no longer considered a viable treatment option. Finally, the addition of the antioxidant N-acetylcysteine to prednisone and azathioprine produced a slight benefit in terms of FVC and DLCO over 12 months of follow up. However, the major benefit appeared to be prevention of the myelotoxicity associated with azathioprine.
Read more about this topic: Idiopathic Pulmonary Fibrosis
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