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.
Pathogenesis
- Pathogenesis of the pulmonary fibrosis characterized by a chronic pattern of excessive fibrosis in the lung tissue which leads to decrease the working space of lungs.[3]
- The core mechanisms of developing pulmonary fibrosis are how the fibrosis take place and collagen metabolism disorder.
Fibrosis
Collagen abnormalities
Genetics
Microscopic pathology
- The microscopic feature associated with idiopathic pulmonary fibrosis is named as "Usual Interstitial Pneumonia (UIP)".[4]
- The histologic findings in UIP include the following:
- Proliferation of mesenchymal cells
- Areas of different fibrosis degree
- Dense deposition of collagen fibers
- Overproduction of extracellular matrix
- Poor differentiated pulmonary architecture
- Honeycomb cysts (subpleural cysts)
Gross pathology
- The most important characteristics of idiopathic pulmonary fibrosis on gross pathology are:[5]
- Inferior lobes fibrosis
- Cobblestone appearance of the pleura
- Airspaces enlargement
- Fibrotic retraction of the airways
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.
- ↑ Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK; et al. (2011). "An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management". Am J Respir Crit Care Med. 183 (6): 788–824. doi:10.1164/rccm.2009-040GL. PMC 5450933. PMID 21471066. Review in: Ann Intern Med. 2011 Jun 21;154(12):JC6-8
- ↑ Gross TJ, Hunninghake GW (2001). "Idiopathic pulmonary fibrosis". N Engl J Med. 345 (7): 517–25. doi:10.1056/NEJMra003200. PMID 11519507.
- ↑ Wolters PJ, Collard HR, Jones KD (2014). "Pathogenesis of idiopathic pulmonary fibrosis". Annu Rev Pathol. 9: 157–79. doi:10.1146/annurev-pathol-012513-104706. PMC 4116429. PMID 24050627.