Hershey Medical Center Hershey Medical Center Geisinger Health System Geisinger Health System Univ of Penn Univ of Penn Temple University Temple University University of Pittsburgh UPMC Simmons Center
PA-IPF Registry home  

The Daniel and Joan Beren
Pennsylvania Idiopathic Pulmonary Fibrosis
State Registry

A project of the University of Pittsburgh, University of Pennsylvania, Penn State Milton S. Hershey Medical Center, Temple University, Geisinger Medical System

PA-IPF Hotline  1-866-922-4IPF (4473)


JOURNALS & CITATIONS

Am J Respir Crit Care Med
Eur Respir J
N Engl J Med
PLoS ONE
Proc Am Thorac Soc
Proc Natl Acad Sci USA
Respir Med

Physician Resources

Proceedings of the American Thoracic Society


Proc Am Thorac Soc. 2006 Jun;3(4):330-8.

Current perspectives on the treatment of idiopathic pulmonary fibrosis

Walter N, Collard HR, King TE, Jr.

Abstract: 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.