A 62-year-old man has progressive dyspnoea over 2 years, bilateral basal crackles, clubbing, and HRCT showing bilateral subpleural basal honeycombing with traction bronchiectasis and minimal ground-glass opacity. Surgical lung biopsy shows UIP pattern. He has no connective tissue disease. What is the FIRST-LINE pharmacological treatment to slow disease progression?

• A Nintedanib or pirfenidone (antifibrotic agents) ✓
• B Oral prednisolone 40 mg daily
• C N-acetylcysteine triple therapy (NAC + prednisolone + azathioprine)
• D Mycophenolate mofetil for immunosuppression

Explanation

This patient has idiopathic pulmonary fibrosis (IPF) with typical UIP pattern. The PANTHER-IPF trial showed that triple therapy (prednisolone + azathioprine + NAC) increased mortality and hospitalisations compared to placebo and was terminated early. Current ATS/ERS/JRS/ALAT 2022 guidelines strongly recommend nintedanib (INPULSIS trials) or pirfenidone (CAPACITY and ASCEND trials) as the only agents shown to slow FVC decline in IPF. Both reduce the rate of FVC decline by ~50% but do not reverse fibrosis.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.