User Tools

Site Tools


interstitial lung disease


  • a diverse group of parenchymal lung diseases which result in damage to the lung interstitium with varying patterns of inflammation and pulmonary fibrosis
  • it is important to distinguish between idiopathic pulmonary fibrosis and other idiopathic interstitial pneumonias with a primary inflammatory pathogenesis as there are major prognostic and therapeutic implications.

clinical diagnosis

  • usually present as SOBOE, persistent dry cough
  • suspect if fine inspiratory creps, fingernail clubbing, or signs of respiratory compromise


  • detailed FH, occupation and medication exposure history
  • CXR
  • high resolution chest CT
  • pulmonary function testing
  • ABG
  • serology for autoimmune disease
  • consider:
    • echocardiogram
    • cardiopulmonary exercise test
    • bronchoscopy
    • surgical lung biopsy
    • overnight sleep study


diseases with known associations

granulomatous diseases


  • lymphangioleiomyomatosis (LAM)
  • histiocytosis X


  • idiopathic pulmonary fibrosis
  • non-specific interstitial pneumonia
  • cryptogenic organising pneumonia
  • acute interstitial pneumonia
  • lymphocytic interstitial pneumonia
  • desquamative interstitial pneumonia
  • respiratory bronchiolitis interstitial lung disease
interstitial_lung.txt · Last modified: 2016/12/03 19:36 (external edit)