Proceedings of the American Thoracic
Proc Am Thorac Soc. 2006 Jun;3(4):330-8.
on the treatment of idiopathic pulmonary fibrosis
Walter N, Collard HR, King TE, Jr.
The clinical course of idiopathic pulmonary fibrosis (IPF) is variable; however,
the long-term survival in IPF is poor. Prednisone has been the mainstay of therapy
since its release for clinical use in 1948. Recently, prednisone combined with azathioprine
or cyclophosphamide has been used. A number of other drug combinations have been
tried with prednisone (e.g., methotrexate, colchicine, penicillamine, or cyclosporine)
but have failed or are not well tolerated by the patient. Few high quality, prospective,
controlled clinical trials have been performed. Thus, there is no good evidence
to support the routine use of any specific therapy in the management of IPF. Additional
large clinical trials are needed to confirm the potential usefulness of the newer
agents (e.g., IFN-gamma1b, pirfenidone, N-acetylcysteine, coumadin, bosentan, or
etanercept). This article examines the body of evidence supporting the current therapies
and reviews the newer agents being tested in patients with IPF.
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