A 45-year-old man with idiopathic pulmonary fibrosis (IPF) has a UIP pattern on HRCT (honeycombing with basal predominance, traction bronchiectasis). FVC 68% predicted, DLCO 52% predicted. Which treatment has been shown to slow FVC decline in IPF?
- A Pirfenidone or nintedanib — anti-fibrotic agents reducing FVC decline by ~50% ✓
- B Oral prednisone 40 mg/day + azathioprine + N-acetylcysteine (triple therapy)
- C Cyclophosphamide IV pulse therapy
- D Long-term high-dose oral N-acetylcysteine alone
Explanation
Pirfenidone (anti-fibrotic, anti-inflammatory, anti-oxidant) and nintedanib (tyrosine kinase inhibitor targeting PDGFR, VEGFR, FGFR) are the only approved disease-modifying therapies for IPF, both reducing the annual rate of FVC decline by approximately 50% in randomised controlled trials (CAPACITY/ASCEND for pirfenidone; INPULSIS for nintedanib). The PANTHER-IPF trial conclusively showed that triple therapy (prednisone + azathioprine + NAC) increased mortality and hospitalisations versus placebo and is now contraindicated in IPF. NAC monotherapy showed no benefit in the PANTHER trial.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.