Idiopathic pulmonary fibrosis : new concepts in pathogenesis and implications for drug therapy.
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.
Horowitz
Jeffrey C
JC
Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Michigan Medical Center, Ann Arbor, Michigan, USA.
Thannickal
Victor J
VJ
D006801:Humans; D054988:Idiopathic Interstitial Pneumonias; D054990:Idiopathic Pulmonary Fibrosis / Q000378:metabolism; D011658:Pulmonary Fibrosis / Q000188:drug therapy