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]

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:
  • 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.
• 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.
• The core mechanisms of developing pulmonary fibrosis are how the fibrosis take place and collagen metabolism disorder.