Idiopathic pulmonary fibrosis

See also Interstitial lung disease

Definition

• Chronic idiopathic interstitial lung disease characterized by a usual interstitial pneumonia on histopathology

Clinical Manifestations

• Fine velcro-like crackles
• Clubbing

Investigations

• CXR showing interstitial pattern
• PFTs showing low DLCO and FVC (i.e. a restrictive pattern)
• High-res CT scan showing a UIP pattern
  • Subpleural/basal
  • Reticulation
  • Honeycombing
  • No incosistent features
• If high-res CT indeterminate, consider biopsy (but 5% mortality from biopsy in ILD)
• CBC, electrolytes
• ANA/ENA, RF, CCP
• Precipitating antibodies

Management

• Smoking cessation
• Vaccines: seasonal influenza and pneumococcal (13 then 23)
• Pulmonary rehab
• Supplemental oxygen
• Medications
  • Consider nintedanib and perfenidone
  • Avoid prednisone and azathioprine
  • NAC not helpful
  • Trial of GERD treatment even if asymptomatic
  • Early antibiotics for respiratory infections