19- IDIOPATHIC PULMONARY FIBROSIS

• Histology

  • Patchy fibrosis
  • Fibroblastic foci
  • Subpleural honeycombing
• Usual age

  Late 60s
• Gender

  More in males
• No known cause
• Physical exam

  • Clubbing in 25-50%
  • Fine end-inspiratory basal crackles
• Diagnosis

  1. Rule out all other causes (ex: do ANA, Rheumatoid factor)

  2. CXR: ground-glass, fibrosis, and honeycomb appearance

  3. High-Resolution CT: Most sensitive, bilateral irregular linear opacities, traction bronchiectasis and honeycombing

  4. Nodules more prominent in the lower lobes

  5. PFT: Restrictive pattern

  6. ABG: hypoxemia with normal PaCO2

  7. Serial lung function is needed to monitor the disease progression
• Treatment


  • The disease is life-limiting and non-curable, and transplantation should be considered
  • Survival without transplant is 5 years
• Supportive care


  1. O2 therapy

  2. Pulmonary rehabilitation

  3. Palliative care
• Pirfenidone


  Anti-fibrotic agent that inhibits collagen synthesis, may slow the rate of decline
• Nintedanib


  TK inhibitor that blocks fibrogenic growth factor
• GERD is present


  It will increase the alveolar epithelial damage
• Avoid immune suppression and steroids