Idiopathic pulmonary fibrosis

From IDWiki

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