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Acute exacerbation of IPF — SCE Respiratory MCQ

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EasyILDAcute exacerbation of IPFSCE Respiratory

A 70-year-old man with known IPF has acute worsening breathlessness over 7 days. CT pulmonary angiography excludes PE but shows new bilateral ground-glass opacity superimposed on established UIP fibrosis. BNP is normal and cultures are negative. What is the most likely diagnosis?

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Correct answer: D — Acute exacerbation of idiopathic pulmonary fibrosis

Acute deterioration with new diffuse ground-glass change on a UIP background after exclusion of PE, infection and heart failure is consistent with acute exacerbation of IPF. Pneumonia and pulmonary oedema are common mimics and must be sought, but the negative work-up makes them less likely. This presentation has high mortality and should prompt early goals-of-care discussion and specialist ILD input.

Reference: ATS/ERS/JRS/ALAT IPF Guideline 2022; NICE IPF guidance