A 38-year-old woman presents with progressive dyspnoea, bilateral interstitial infiltrates on CT showing ground-glass opacity with reticulation, honeycombing predominantly in the basal and subpleural distribution, and usual interstitial pneumonia (UIP) pattern confirmed by HRCT. Which antifibrotic therapy has demonstrated slowing of FVC decline in IPF?
- A Pirfenidone or nintedanib ✓
- B Azathioprine plus N-acetylcysteine
- C Prednisolone plus cyclophosphamide
- D Rituximab as first-line immunosuppression
Explanation
Both pirfenidone (ASCEND, CAPACITY trials) and nintedanib (INPULSIS trial) have demonstrated reduction in the rate of FVC decline by approximately 50% in idiopathic pulmonary fibrosis (IPF), and are approved antifibrotic therapies. Pirfenidone is a pleiotropic antifibrotic; nintedanib inhibits PDGFR, VEGFR, and FGFR tyrosine kinases. The 2012 PANTHER trial showed triple therapy (prednisolone + azathioprine + N-acetylcysteine) was harmful in IPF, increasing mortality and hospitalizations. Immunosuppression alone has no role in IPF; it is used for other fibrotic ILDs like hypersensitivity pneumonitis or CTD-ILD.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.