A 45-year-old non-smoking woman presents with progressive dyspnea and bilateral basal crackles. CT chest shows subpleural honeycombing with traction bronchiectasis predominantly at the bases. PFTs: FVC 60% predicted, DLCO 45% predicted, FEV1/FVC 0.82. Diagnosis of IPF is established. Which medication has been shown to slow FVC decline in IPF?
- A Nintedanib or pirfenidone ✓
- B Prednisolone + azathioprine + N-acetylcysteine
- C Mycophenolate mofetil
- D Cyclophosphamide
Correct answer: A. Nintedanib or pirfenidone
Explanation
Two antifibrotic agents, nintedanib (tyrosine kinase inhibitor targeting PDGFR, FGFR, VEGFR) and pirfenidone (anti-fibrotic, anti-inflammatory mechanism), have each been shown in Phase III RCTs (INPULSIS and ASCEND trials respectively) to slow the annual decline in FVC by approximately 50% compared to placebo in IPF. Both are ATS/ERS/JRS/ALAT 2022 conditional recommendations. The PANTHER-IPF trial specifically showed that prednisolone + azathioprine + NAC worsened outcomes vs. placebo. Mycophenolate may be used in HP or CTD-ILD, not IPF.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.