A 44-year-old man is diagnosed with IPF (idiopathic pulmonary fibrosis) based on HRCT and clinical criteria. He has FVC 72% predicted and DLCO 58%. According to current ATS/ERS guidelines, the antifibrotic drug that inhibits TGF-β1 signaling and PDGF/VEGF receptor tyrosine kinases, approved for slowing FVC decline in IPF, is:
- A Pirfenidone
- B N-acetylcysteine
- C Sildenafil
- D Nintedanib ✓
Explanation
Nintedanib (Ofev) is a triple kinase inhibitor targeting PDGFR-α/β, VEGFR-1/2/3, and FGFR-1/2/3, all of which are involved in fibrotic pathways. It also inhibits TGF-β-induced fibroblast activation indirectly. The INPULSIS-1 and INPULSIS-2 trials showed nintedanib reduced annual FVC decline by ~125 mL/year versus placebo. Pirfenidone acts on TGF-β1 and TNF-α, reducing fibroblast proliferation (ASCEND trial). Both are approved for IPF and slow disease progression without reversing fibrosis. N-acetylcysteine showed no benefit in the PANTHER-IPF trial. Sildenafil is used for IPF-related pulmonary hypertension but does not slow fibrosis.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.