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

Genetics

Microscopic pathology

Gross pathology

References

  1. van der Rest M, Garrone R (1991). "Collagen family of proteins". FASEB J. 5 (13): 2814–23. PMID 1916105.
  2. 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.
  3. 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

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