Idiopathic pulmonary fibrosis pathophysiology: Difference between revisions

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** Inferior lobes fibrosis  
** Inferior lobes fibrosis  
** Cobblestone appearance of the pleura  
** Cobblestone appearance of the pleura  
** Honeycomb appearance of the lung 
** Airspaces enlargement  
** Airspaces enlargement  
** Fibrotic retraction of the airways
** Fibrotic retraction of the airways

Revision as of 15:15, 15 February 2018

Idiopathic pulmonary fibrosis Microchapters

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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
    • Honeycomb appearance of the lung
    • Airspaces enlargement
    • Fibrotic retraction of the airways
Honeycomb appearance of a fibrotic lung.
Case courtesy of A.Prof Frank Gaillard, <a href="https://radiopaedia.org/">Radiopaedia.org</a>. From the case <a href="https://radiopaedia.org/cases/8621">rID: 8621</a> via www.radiopaedia.org

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
  4. Gross TJ, Hunninghake GW (2001). "Idiopathic pulmonary fibrosis". N Engl J Med. 345 (7): 517–25. doi:10.1056/NEJMra003200. PMID 11519507.
  5. 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.

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