A 45-year-old non-smoker woman is diagnosed with idiopathic pulmonary fibrosis (IPF). CT shows bilateral basal-predominant honeycombing with traction bronchiectasis. PFTs show FVC 68%, DLCO 52%. Which treatment has been shown to slow FVC decline in IPF?

• A High-dose prednisolone
• B Nintedanib or pirfenidone ✓
• C N-acetylcysteine triple therapy (prednisolone + azathioprine + NAC)
• D Rituximab for autoimmune hypothesis

Explanation

Nintedanib (a tyrosine kinase inhibitor targeting FGFR, PDGFR, VEGFR) and pirfenidone (an antifibrotic with TGF-β inhibition) are the only approved disease-modifying agents for IPF. Both reduce the rate of FVC decline by approximately 50% without reversing established fibrosis. The PANTHER-IPF trial showed that triple therapy (prednisone + azathioprine + NAC) increased mortality and hospitalizations and is now contraindicated. High-dose steroids alone are ineffective and harmful 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.