Fibrosing lung disease

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

Reference:

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