A 54-year-old woman presents with progressive dyspnea for 2 years. HRCT shows bilateral basal, subpleural reticulation with honeycombing and traction bronchiectasis. Bronchoalveolar lavage shows no significant lymphocytosis. Multidisciplinary team (MDD) diagnosis is usual interstitial pneumonia (UIP) pattern, consistent with IPF. FVC is 72% predicted. Which treatment has been shown to slow FVC decline in IPF?
- A Pirfenidone or nintedanib ✓
- B Oral prednisolone + azathioprine + N-acetylcysteine (ACE-triple therapy)
- C Cyclophosphamide
- D Colchicine
Correct answer: A. Pirfenidone or nintedanib
Explanation
Both pirfenidone (antifibrotic) and nintedanib (tyrosine kinase inhibitor targeting PDGFR, VEGFR, FGFR) reduce the annual rate of FVC decline in IPF by approximately 50% and are the only disease-modifying agents approved for IPF. The CAPACITY/ASCEND trials established pirfenidone and INPULSIS trials established nintedanib. The PANTHER-IPF trial showed that triple therapy (prednisone + azathioprine + NAC) actually increased mortality and hospitalizations compared to placebo and is now contraindicated. Cyclophosphamide has no benefit in IPF.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.