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Pulmonary hypertension associated with ILD — SCE Respiratory MCQ

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HardPulmonary Vascular DiseasePulmonary hypertension associated with ILDSCE Respiratory

A 70-year-old man with idiopathic pulmonary fibrosis has increasing dyspnoea. Echo suggests pulmonary hypertension and CT shows extensive fibrosis. ABG shows PaO2 7.4 kPa and PaCO2 4.8 kPa. Right heart catheterisation confirms pre-capillary pulmonary hypertension. What is the most likely underlying cause?

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Correct answer: D — Group 3 pulmonary hypertension due to fibrotic lung disease and hypoxaemia

Established IPF with extensive fibrosis and hypoxaemia makes group 3 pulmonary hypertension the best explanation. Group 1 PAH is less likely when substantial parenchymal lung disease is present, and group 2 disease would require left heart pathology or elevated wedge pressure. Acute PE and portopulmonary hypertension are not supported by the chronic IPF-centred data.

Reference: ESC/ERS Pulmonary Hypertension Guideline 2022; NICE CG163