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SSc Hypertension Assessment — SCE Rheumatology MCQ

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ModerateSystemic SclerosisSSc Hypertension AssessmentSCE Rheumatology

A 50-year-old woman diagnosed 14 months ago with diffuse cutaneous systemic sclerosis, positive for anti-RNA polymerase III antibodies, attends urgently with a two-day headache. Her home readings are usually around 112/70 mmHg and her baseline serum creatinine three weeks ago was 58 micromol/L. Today, repeated seated measurements are 172/104 mmHg. Serum creatinine is 79 micromol/L (reference 45–90), urinalysis shows trace protein, haemoglobin 118 g/L and platelets 148 x 10^9/L. There has been no change to her medicines and she takes no glucocorticoids. Which interpretation and immediate action are most appropriate?

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Correct answer: A — Evolving scleroderma renal crisis; start an ACE inhibitor now with urgent specialist monitoring

This is evolving scleroderma renal crisis (SRC) and must be treated as such. The decisive features are abrupt, confirmed severe hypertension in a patient with early diffuse cutaneous disease and anti-RNA polymerase III antibodies — the highest-risk phenotype — with a creatinine that has risen roughly 35% from her individual baseline despite remaining inside the laboratory reference interval. UK Scleroderma Study Group guidance defines SRC by acute severe hypertension with acute kidney injury and mandates prompt ACE inhibitor therapy (titrated, continued long term) with close monitoring of blood pressure, renal function, urinalysis, full blood count and blood film for microangiopathy. Essential and white-coat hypertension cannot explain the symptomatic acute rise plus falling renal function; deferring treatment for ambulatory monitoring risks irreversible renal failure within days. Renal artery stenosis is far less likely in this phenotype and imaging must never delay ACE inhibition. Drug-induced hypertension is untenable with no medication change and no steroid exposure.

Reference: Lynch BM, Stern EP, Ong V, et al. UK Scleroderma Study Group (UKSSG) guidelines on the diagnosis and management of scleroderma renal crisis. Clin Exp Rheumatol 2016 — diagnosis and ACE inhibitor management sections. https://pubmed.ncbi.nlm.nih.gov/27749244/