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Fibrosing lung disease

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

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Pulmonary fibrosis is a condition in which there is diffuse fibrosis of lung parenchyma with a resultant impairment of gas transfer and ventilation-perfusion mismatching.

Fibrosis within the lung may be:

  • replacement fibrosis, secondary to disorders such as:
    • infarction
    • pneumonia
    • tuberculosis

  • focal fibrosis, in response to irritants such as:
    • silica
    • coal dust

  • interstitial fibrosis:
    • cryptogenic (idiopathic) fibrosing alveolitis
    • extrinsic allergic alveolitis

Interstitial lung disease (ILD) consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure (1):

  • most common forms of ILD are idiopathic pulmonary fibrosis (IPF), which accounts for approximately one-third of all cases of ILD, hypersensitivity pneumonitis, accounting for 15% of ILD cases, and connective tissue disease (CTD), accounting for 25% of ILD cases
  • typically presents with dyspnea on exertion
  • approximately 30% of patients with ILD report cough


  1. Maher TM. Interstitial Lung Disease: A Review. JAMA. Published online April 22, 2024. doi:10.1001/jama.2024.3669

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