Idiopathic Pulmonary Fibrosis (IPF)

IPF is a chronic, progressive fibrosing interstitial pneumonia of unknown cause, occurring primarily in older adults. It is the most common idiopathic interstitial pneumonia and carries a median survival of 3–5 years from diagnosis. The pathologic pattern is usual interstitial pneumonia (UIP).

• 1Diagnosis requires: clinical context (age >60, male, smoker) + typical UIP on HRCT ± surgical biopsy
• 2Typical UIP HRCT: bilateral basal honeycombing ± traction bronchiectasis, minimal GGO
• 3Antifibrotic therapy: pirfenidone (ASCEND) or nintedanib (INPULSIS) — both slow FVC decline
• 4Triple therapy (prednisone + AZA + NAC) is HARMFUL — PANTHER-IPF trial
• 5Acute exacerbation of IPF: rapid deterioration, new bilateral GGO on background UIP — high mortality
• 6Lung transplant: only intervention that improves survival

• BAL lymphocytes >20% argue against IPF — consider HP or NSIP
• Velcro crackles + clubbing = classic IPF exam finding
• Pirfenidone: main side effect = photosensitivity, GI (nausea)
• Nintedanib: main side effect = diarrhea (60%), hepatotoxicity
• Antifibrotics slow progression but do not reverse fibrosis