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Acute exacerbation of IPF — SCE Respiratory MCQ

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HardILDAcute exacerbation of IPFSCE Respiratory

A 76-year-old man with known IPF deteriorates over 10 days with new severe hypoxaemia. CT pulmonary angiography shows no embolus but demonstrates new bilateral ground-glass opacities superimposed on established UIP fibrosis. CRP is 18 mg/L, viral PCR is negative and echocardiography shows no left heart failure. What is the most likely diagnosis?

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Correct answer: E — Acute exacerbation of idiopathic pulmonary fibrosis

Rapid respiratory deterioration with new bilateral ground-glass change on UIP background after excluding infection, PE and heart failure is typical of acute exacerbation of IPF. Pneumonia and pulmonary oedema are tempting but the investigations do not support them, and aspergillosis is usually subacute in cavities. The clinical pearl is that acute IPF exacerbation is a diagnosis of exclusion and carries high mortality.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/respiratory-conditions