Mechanisms of pulmonary fibrosis
Tissue injury evokes highly conserved, tightly regulated inflammatory responses and less well-understood host repair responses. Both inflammation and repair involve the recruitment, activation, apoptosis, and eventual clearance of key effector cells. In this review, we propose the concept of pulmona...
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Veröffentlicht in: | Annual review of medicine 2004-01, Vol.55 (1), p.395-417 |
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creator | Thannickal, Victor J Toews, Galen B White, Eric S Lynch, 3rd, Joseph P Martinez, Fernando J |
description | Tissue injury evokes highly conserved, tightly regulated inflammatory responses and less well-understood host repair responses. Both inflammation and repair involve the recruitment, activation, apoptosis, and eventual clearance of key effector cells. In this review, we propose the concept of pulmonary fibrosis as a dysregulated repair process that is perpetually "turned on" even though classical inflammatory pathways may be dampened or "switched off." Significant regional heterogeneity, with varied histopathological patterns of inflammation and fibrosis, has been observed in individual patients with idiopathic pulmonary fibrosis. We discuss environmental factors and host response factors, such as genetic susceptibility and age, that may influence these varied manifestations. Better understanding of the mechanisms of lung repair, which include alveolar reepithelialization, myofibroblast differentiation/activation, and apoptosis, should offer more effective therapeutic options for progressive pulmonary fibrosis. |
doi_str_mv | 10.1146/annurev.med.55.091902.103810 |
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Both inflammation and repair involve the recruitment, activation, apoptosis, and eventual clearance of key effector cells. In this review, we propose the concept of pulmonary fibrosis as a dysregulated repair process that is perpetually "turned on" even though classical inflammatory pathways may be dampened or "switched off." Significant regional heterogeneity, with varied histopathological patterns of inflammation and fibrosis, has been observed in individual patients with idiopathic pulmonary fibrosis. We discuss environmental factors and host response factors, such as genetic susceptibility and age, that may influence these varied manifestations. 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subjects | Cystic fibrosis Genetics Humans Lung - immunology Lung - pathology Lung - physiopathology Lungs Medical research Pulmonary Fibrosis - etiology Pulmonary Fibrosis - pathology Pulmonary Fibrosis - physiopathology |
title | Mechanisms of pulmonary fibrosis |
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