A 52-year-old non-smoker woman presents with progressive dyspnoea and bibasilar crackles. HRCT shows honeycombing with traction bronchiectasis predominantly in the lower lobes, subpleural distribution. Spirometry: FVC 62% predicted, DLCO 48% predicted. Lung biopsy shows UIP pattern. The MOST appropriate antifibrotic therapy is:
- A N-acetylcysteine alone
- B Prednisolone 60 mg/day
- C Azathioprine and prednisolone combination
- D Pirfenidone ✓
Explanation
Idiopathic pulmonary fibrosis (IPF) with UIP pattern on HRCT/biopsy is treated with antifibrotic agents: pirfenidone (an anti-inflammatory, anti-fibrotic agent inhibiting TGF-beta) or nintedanib (a tyrosine kinase inhibitor targeting PDGFR, FGFR, VEGFR). Both slow FVC decline by approximately 50% per year and reduce acute exacerbation risk. The PANTHER-IPF trial showed that N-acetylcysteine alone offered no benefit. Immunosuppression (prednisolone ± azathioprine) was shown harmful in IPF in PANTHER-IPF — not superior and associated with increased hospitalisation and death.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.