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Systemic sclerosis-associated ILD — SCE Respiratory MCQ

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HardILDSystemic sclerosis-associated ILDSCE Respiratory

A 49-year-old woman with systemic sclerosis has new exertional dyspnoea. FVC is 71% predicted, TLCO 38% predicted and HRCT shows basal ground-glass change with fine reticulation. NT-proBNP is normal and echocardiography does not suggest pulmonary hypertension. What is the most appropriate management?

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Correct answer: E — Discuss immunosuppression for systemic sclerosis-associated ILD

Basal inflammatory-fibrotic changes with reduced gas transfer in systemic sclerosis are consistent with systemic sclerosis-associated ILD, for which specialist ILD discussion and immunosuppression are appropriate. A preserved or near-preserved FVC does not exclude clinically important disease when TLCO and symptoms are abnormal. PE and pneumonia are not supported by the chronic pattern and HRCT appearances.

Reference: ERS/EULAR Systemic Sclerosis ILD Guidance; NICE NG163