In the management of idiopathic pulmonary fibrosis (IPF), the ATS/ERS/JRS/ALAT 2022 guidelines give a 'conditional recommendation for' (previously 'weak for') which antifibrotic therapy?
- A High-dose N-acetylcysteine triple therapy
- B Azathioprine plus prednisone
- C Nintedanib (a FGFR/VEGFR/PDGFR tyrosine kinase inhibitor) ✓
- D Warfarin anticoagulation for subclinical PE in IPF
Correct answer: C. Nintedanib (a FGFR/VEGFR/PDGFR tyrosine kinase inhibitor)
Explanation
Both nintedanib and pirfenidone are now conditionally recommended by ATS/ERS/JRS/ALAT 2022 guidelines for IPF as they slow decline in FVC by ~50% compared to placebo (demonstrated in INPULSIS and CAPACITY/ASCEND trials respectively). Nintedanib targets intracellular kinases (FGFR, VEGFR, PDGFR) involved in fibroblast signalling. N-acetylcysteine triple therapy was shown to be HARMFUL in the PANTHER trial. Azathioprine + prednisone also increased mortality in PANTHER. Warfarin was shown to increase mortality in IPF (ACE-IPF trial).
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.