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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Bibliographic Details
Published in:Annual review of medicine 2004-01, Vol.55 (1), p.395-417
Main Authors: Thannickal, Victor J, Toews, Galen B, White, Eric S, Lynch, 3rd, Joseph P, Martinez, Fernando J
Format: Article
Language:English
Subjects:
Cystic fibrosis
Genetics
Humans
Lung - immunology
Lung - pathology
Lung - physiopathology
Lungs
Medical research
Pulmonary Fibrosis - etiology
Pulmonary Fibrosis - pathology
Pulmonary Fibrosis - physiopathology
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Summary: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.
ISSN:0066-4219
1545-326X
DOI:10.1146/annurev.med.55.091902.103810