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Idiopathic Pulmonary Fibrosis

New Concepts in Pathogenesis and Implications for Drug Therapy

  • Review Article
  • Published:
Treatments in Respiratory Medicine

Abstract

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, and usually fatal pulmonary disease for which there are no proven drug therapies. Anti-inflammatory and immunosuppressive agents have been largely ineffective. The precise relationship of IPF to other idiopathic interstitial pneumonias (IIPs) is not known, despite the observation that different histopathologic patterns of IIP may coexist in the same patient. We propose that these different histopathologic ‘reaction’ patterns may be determined by complex interactions between host and environmental factors that alter the local alveolar milieu. Recent paradigms in IPF pathogenesis have focused on dysregulated epithelial-mesenchymal interactions, an imbalance in TH1/TH2 cytokine profile and potential roles for aberrant angiogenesis. In this review, we discuss these evolving concepts in disease pathogenesis and emerging therapies designed to target pro-fibrogenic pathways in IPF.

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Table I

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Notes

  1. 1 The use of trade names is for product identification purposes only and does not imply endorsement.

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Acknowledgments

This work was supported, in part, by National Institutes of Health grants K08 HL081059 (to J.C.H.); R01 HL67967 and P50 HL74024 (to V.J.T.). The authors do not have any potential conflicts of interest that are directly relevant to the contents of this article.

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  1. Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Michigan Medical Center, 6301 MSRB III, 1150 W. Medical Center Dr, Ann Arbor, Michigan, 48109, USA

    Jeffrey C. Horowitz &  Victor J. Thannickal

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Correspondence to Victor J. Thannickal.

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Horowitz, J.C., Thannickal, V.J. Idiopathic Pulmonary Fibrosis. Treat Respir Med 5, 325–342 (2006). https://doi.org/10.2165/00151829-200605050-00004

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