A 70-year-old man presents with progressive dyspnea and dry cough for 2 years. He is a never-smoker. HRCT shows bilateral basal and subpleural honeycombing with traction bronchiectasis. Spirometry shows a restrictive pattern with FVC 58% and DLCO 45% of predicted. Bronchoalveolar lavage shows no lymphocytosis. Surgical lung biopsy confirms usual interstitial pneumonia (UIP) pattern. Which of the following best describes the pharmacotherapy with proven benefit in this condition?
- A Nintedanib or pirfenidone to slow FVC decline ✓
- B High-dose oral prednisolone to reverse fibrosis
- C N-acetylcysteine monotherapy for antioxidant effect
- D Azathioprine plus low-dose prednisolone
Explanation
Idiopathic pulmonary fibrosis (IPF) with a biopsy-confirmed UIP pattern is treated with antifibrotic agents. Both nintedanib (a tyrosine kinase inhibitor) and pirfenidone reduce the annual rate of FVC decline by approximately 50%, thereby slowing disease progression; neither reverses established fibrosis. High-dose corticosteroids worsen outcomes in IPF and are contraindicated. The PANTHER-IPF trial showed that N-acetylcysteine monotherapy offers no benefit over placebo. The combination of azathioprine + prednisolone + N-acetylcysteine was shown in PANTHER-IPF to increase mortality and was abandoned.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.