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American Journal of Respiratory and Critical Care Medicine Vol 166. pp. 236-246, (2002)
© 2002 American Thoracic Society

NHBLI WORKSHOP SUMMARY

Future Research Directions in Idiopathic Pulmonary Fibrosis

Summary of a National Heart, Lung, and Blood Institute Working Group

Ronald G. Crystal, Peter B. Bitterman, Brooke Mossman, Marvin I. Schwarz, Dean Sheppard, Laura Almasy, Harold A. Chapman, Scott L. Friedman, Talmadge E. King, Jr., Leslie A. Leinwand, Lance Liotta, George R. Martin, David A. Schwartz, Gregory S. Schultz, Carston R. Wagner and Robert A. Musson

Division of Pulmonary and Critical Care Medicine, Weill Medical College of Cornell University, New York, New York; Department of Medicine, University of Minnesota Medical School, Minneapolis, Minnesota; Department of Pathology, University of Vermont, Burlington, Vermont; Pulmonary Sciences and Critical Care Medicine, University of Colorado Health Sciences Center, Denver, Colorado; Department of Medicine, University of California, San Francisco; Department of Genetics, Southwest Foundation for Biomedical Research, San Antonio, Texas; Pulmonary and Critical Care Division, University of California at San Francisco, San Francisco, California; Division of Liver Diseases, Mount Sinai School of Medicine, New York, New York; Division of Pulmonary and Critical Care Medicine, Department of Medicine, San Francisco General Hospital, University of California at San Francisco, San Francisco, California; Department of Molecular, Cellular, and Developmental Biology, University of Colorado, Boulder, Colorado; Cell and Cancer Biology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland; FibroGen Inc., San Francisco, California; Department of Medicine, Duke University, Durham, North Carolina; Department of Obstetrics and Gynecology, University of Florida, Gainesville, Florida; Department of Medicinal Chemistry, University of Minnesota, Minneapolis, Minnesota; and Division of Lung Diseases, National Heart, Lung, and Blood Institute, Bethesda, Maryland

Correspondence and requests for reprints should be addressed to Robert A. Musson, Room 7126, MSC 7922, 6701 Rockledge Drive, Bethesda, MD 20892. E-mail: rmusson{at}nih.gov

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is an insidious inflammatory fibroproliferative disease whose cause and course before diagnosis are unknown, and for which existing treatments are of limited benefit. The National Heart, Lung, and Blood Institute convened a working group to develop specific recommendations for future IPF research. Inflammatory and immune processes are involved in IPF pathogenesis, and current therapeutic strategies are aimed at suppressing the inflammation. Recent data suggest that the molecular processes underlying the fibrogenesis may provide new opportunities for therapeutic intervention. Specific areas of future research recommended by the working group include studies to elucidate the etiology of IPF, to develop novel diagnostic techniques and molecular diagnostics, to establish a program for identification of molecular targets for IPF treatment and identification and generation of agonists or antagonists that inhibit fibrogenesis, to foster investigations that couple the use of new technologies (e.g., laser capture microdissection, microarrays, and mass spectroscopic analysis of proteins) with data from the human genome project, to establish a national consortium of Clinical Centers of Excellence to conduct coordinated clinical and laboratory studies of well-characterized patients and patient-derived materials, and to stimulate research to develop animal models of persistent and progressive pulmonary fibrosis for evaluation of new intervention approaches.

Key Words: lung diseases, interstitial • pulmonary fibrosis • National Institutes of Health (United States)




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