A 45-year-old woman has progressive dyspnoea on exertion, dry cough, and bibasal fine crepitations over 18 months. HRCT shows honeycombing in subpleural and basal distribution, with traction bronchiectasis. Surgical lung biopsy shows usual interstitial pneumonia (UIP) pattern. Which management is most appropriate per ATS/ERS 2022 IPF guidelines?

• A Oral prednisolone + azathioprine + N-acetylcysteine (triple therapy)
• B Nintedanib or pirfenidone (antifibrotic therapy) ✓
• C Rituximab + steroids
• D Cyclosporine A

Explanation

Nintedanib (INPULSIS trial) and pirfenidone (ASCEND trial) are the only two antifibrotic agents proven to slow the annual decline in FVC in IPF and are recommended by ATS/ERS/JRS/ALAT 2022 guidelines. The PANTHER-IPF trial established that the triple therapy of prednisone + azathioprine + N-acetylcysteine was harmful (increased mortality and hospitalisations) and is now contraindicated. The choice between the two antifibrotics depends on patient comorbidities and tolerability.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.