see also:

• respiratory medicine

• 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.

• 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

• connective tissue (CT) disorders
• drugs
• occupational exposure - pneumoconiosis

• sarcoidosis
• hypersensitivity pneumonitis

• 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