A 40-year-old non-smoker woman presents with 8 months of progressive dyspnoea. HRCT chest shows bilateral basal subpleural honeycombing with traction bronchiectasis. Surgical lung biopsy confirms a UIP pattern. Spirometry: FVC 58%, DLCO 45%. ANA is weakly positive at 1:80, speckled. What is the diagnosis and appropriate antifibrotic therapy?
- A Idiopathic pulmonary fibrosis (IPF); treat with nintedanib or pirfenidone ✓
- B Hypersensitivity pneumonitis; treat with corticosteroids and antigen avoidance
- C Connective tissue disease-ILD; treat with mycophenolate as antifibrotics are contraindicated
- D Non-specific interstitial pneumonia (NSIP); treat with corticosteroids and immunosuppression
Correct answer: A. Idiopathic pulmonary fibrosis (IPF); treat with nintedanib or pirfenidone
Explanation
UIP pattern on HRCT with honeycombing plus traction bronchiectasis in the correct clinical setting (progressive fibrotic ILD, no identifiable cause) is diagnostic of IPF per ATS/ERS/JRS/ALAT 2022 guidelines. A low-titre ANA does not exclude IPF. Nintedanib and pirfenidone are both approved antifibrotic agents that slow FVC decline (INPULSIS and ASCEND trials). NSIP shows more ground-glass opacity and less honeycombing.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.