Idiopathic pulmonary fibrosis pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]
Overview
Pathophysiology
Normal lung tissue
- Lungs are composed normally of extracellular collagen which allows the lungs to exert their breathing efforts.
- Different collagen types in the lung include the following:[1]
- Type 1 and type 3 compose most of the lung tissue
- Type 2 is the main component of the cartilage of the main bronchi
- Type 4 forms the basement membrane
- Type 5 forms the interstitial tissue
- Normally, collagen is degraded and produced regularly to preserve the normal lung tissue.[2]
- Collagen is produced by fibroblasts which also can degrade some of the collagen produced.
- Metalloproteinases produced by fibroblasts, neutrophils, and macrophages have the main role in degrading collagen.
Genetics
Microscopic pathology
Gross pathology
References
- ↑ van der Rest M, Garrone R (1991). "Collagen family of proteins". FASEB J. 5 (13): 2814–23. PMID 1916105.
- ↑ Laurent GJ (1982). "Rates of collagen synthesis in lung, skin and muscle obtained in vivo by a simplified method using [3H]proline". Biochem J. 206 (3): 535–44. PMC 1158621. PMID 7150261.