Systemic sclerosis renal crisis — SCE Rheumatology MCQ

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ModerateConnective tissue diseasesSystemic sclerosis renal crisisSCE Rheumatology

A 45-year-old man with early diffuse systemic sclerosis presents with headache and visual disturbance. His blood pressure is 198/112 mmHg and skin thickening is rapidly progressive. Serum creatinine has doubled from baseline, and urinalysis shows mild proteinuria. What is the most appropriate immediate management?

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Correct answer: C — Start an ACE inhibitor urgently

This presentation is scleroderma renal crisis: early diffuse systemic sclerosis with abrupt severe hypertension, neurological symptoms and acute kidney injury. Start an ACE inhibitor urgently, with close inpatient monitoring and dose titration to control blood pressure. A rising creatinine does not justify withholding ACE inhibition in this setting. High-dose glucocorticoids are not acute treatment and increase the risk of renal crisis. A calcium-channel blocker may be added for persistent hypertension but is inadequate as sole initial therapy. Rituximab has no role in the immediate management of renal crisis. Renal biopsy may be considered when the diagnosis is uncertain, especially in atypical presentations, but should not delay ACE-inhibitor treatment here.

Reference: Denton CP et al. UK Scleroderma Study Group (UKSSG) guidelines on the diagnosis and management of scleroderma renal crisis, 2016. https://pubmed.ncbi.nlm.nih.gov/27749244/