A patient with proven IPF (UIP on HRCT, excluded other causes) has FVC 68% predicted, DLCO 58%. What is the first-line antifibrotic therapy?
- A N-acetylcysteine high dose
- B Prednisolone plus azathioprine
- C Nintedanib or pirfenidone ✓
- D Mycophenolate mofetil monotherapy
Correct answer: C. Nintedanib or pirfenidone
Explanation
Both nintedanib (INPULSIS trial) and pirfenidone (ASCEND, CAPACITY trials) are approved antifibrotic agents that slow FVC decline in IPF and are considered equivalent first-line options per ATS/ERS guidelines. The triple immunosuppressive regimen (prednisolone + azathioprine + NAC) was shown to be harmful in IPF in the PANTHER-IPF trial and is contraindicated. NAC monotherapy showed no benefit in PANTHER-IPF. MMF has no proven efficacy in 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.