Idiopathic pulmonary fibrosis medical therapy

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Medical Therapy

There is currently no consensus on the treatment of IPF. Hence, none of what follows should be taken as specific advice regarding therapy, as the latter is a decision that must be made on a case-by-case basis in individual patients.

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.

Small early studies demonstrated that the combination of prednisone with either cyclophosphamide or azathioprine over many months had very modest, if any, beneficial effect in IPF, and were associated with substantial adverse effects (predominantly myelotoxicity). Other treatments studied have included interferon gamma-1b and the antifibrotic agent pirfenidone. While neither drug has been shown to have substantial benefits over time, both are currently being studied in patients with IPF. 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.