Idiopathic Pulmonary Fibrosis : New Concepts in Pathogenesis and Implications for Drug Therapy

Overview

Journal Treat Respir Med

Date 2006 Aug 25

PMID 16928146

Citations 18

Authors

Jeffrey C Horowitz

Victor J Thannickal

Affiliations

Soon will be listed here.

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 T(H)1/T(H)2 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.

Citing Articles

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p53 and Myofibroblast Apoptosis in Organ Fibrosis.

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The Challenge of Long-Term Cultivation of Human Precision-Cut Lung Slices.

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Regorafenib-Attenuated, Bleomycin-Induced Pulmonary Fibrosis by Inhibiting the TGF-β1 Signaling Pathway.

Li X, Ma L, Huang K, Wei Y, Long S, Liu Q Int J Mol Sci. 2021; 22(4).

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Angiotensin Receptor Blockers and Subclinical Interstitial Lung Disease: The MESA Study.

Gannon W, Anderson M, Podolanczuk A, Kawut S, Michos E, Cottin V Ann Am Thorac Soc. 2019; 16(11):1451-1453.

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