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, Leslie A. Leinwand, Lance Liotta, George R. Martin, David A. Schwartz, Gregory S. Schultz, Carston R. Wagner, Robert A. Musson

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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.

Original languageEnglish
Pages (from-to)236-246
Number of pages11
JournalAmerican Journal of Respiratory and Critical Care Medicine
Volume166
Issue number2
DOIs
Publication statusPublished - 15 Jul 2002

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Keywords

  • Lung diseases, interstitial
  • National Institutes of Health (United States)
  • Pulmonary fibrosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine

Cite this

Crystal, R. G., Bitterman, P. B., Mossman, B., Schwarz, M. I., Sheppard, D., Almasy, L., Chapman, H. A., Friedman, S. L., King, T. E., Leinwand, L. A., Liotta, L., Martin, G. R., Schwartz, D. A., Schultz, G. S., Wagner, C. R., & Musson, R. A. (2002). Future research directions in idiopathic pulmonary fibrosis: Summary of a National Heart, Lung, and Blood Institute Working Group. American Journal of Respiratory and Critical Care Medicine, 166(2), 236-246. https://doi.org/10.1164/rccm.2201069