A 52-year-old non-smoker woman presents with 2 years of progressive exertional dyspnea. HRCT shows bilateral basal, subpleural honeycombing with traction bronchiectasis and minimal ground glass. Surgical lung biopsy shows usual interstitial pneumonia (UIP) pattern. Spirometry: FVC 58% predicted, DLCO 45% predicted. What is the current first-line pharmacological treatment?

• A High-dose prednisolone plus azathioprine
• B Nintedanib or pirfenidone (antifibrotic therapy) ✓
• C N-acetylcysteine (NAC) triple therapy
• D Cyclophosphamide monthly IV pulses

Explanation

Idiopathic pulmonary fibrosis (IPF) with UIP pattern on HRCT/biopsy is treated with antifibrotic agents — pirfenidone (CAPACITY and ASCEND trials) and nintedanib (INPULSIS trial) — both of which slow the rate of FVC decline by approximately 50% and reduce acute exacerbations. The PANTHER-IPF trial demonstrated that the triple combination of prednisone + azathioprine + NAC was harmful (increased mortality and hospitalizations). Current ATS/ERS/JRS/ALAT 2022 guidelines give a strong recommendation for antifibrotic therapy 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.