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Scleroderma renal crisis requiring renal replacement therapy — SCE Rheumatology MCQ

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ModerateRheumatology EmergenciesScleroderma renal crisis requiring renal replacement therapySCE Rheumatology

A 39-year-old woman with rapidly progressive diffuse cutaneous systemic sclerosis is admitted with headache, breathlessness and blurred vision. Non-Raynaud disease began 14 months ago, and anti-RNA polymerase III antibodies are present. Her blood pressure is 224/126 mmHg, compared with 112/70 mmHg at her previous clinic visit. Serum creatinine is 278 micromol/L, having been 68 micromol/L 3 weeks earlier. Platelets are 96 × 10^9/L, lactate dehydrogenase is elevated and the blood film contains occasional schistocytes. Urinalysis shows blood 2+ and protein 2+. There is no focal neurological deficit. ADAMTS13 activity has been requested. Scleroderma renal crisis is diagnosed and captopril commenced. Twenty-four hours later, her blood pressure is 154/92 mmHg, but she is oliguric and creatinine has risen to 416 micromol/L. She develops pulmonary oedema despite intravenous furosemide, and arterial pH remains 7.12 despite initial medical treatment. Which management plan is most appropriate?

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Correct answer: A — Continue captopril and initiate urgent renal replacement therapy

The phenotype strongly supports scleroderma renal crisis: early rapidly progressive diffuse cutaneous disease, anti-RNA polymerase III positivity, abrupt severe hypertension and rapidly progressive acute kidney injury. Mild thrombocytopenia, schistocytes and elevated lactate dehydrogenase reflect the thrombotic microangiopathy that commonly accompanies renal crisis and do not, in this context, establish immune thrombotic thrombocytopenic purpura. Pulmonary oedema unresponsive to medical management and persistent severe metabolic acidosis are immediate indications for renal replacement therapy. Treatment should not be delayed according to the creatinine trajectory or an arbitrary observation period. Captopril should be continued. UK specialty guidance recommends ongoing ACE-inhibitor therapy in scleroderma renal crisis even when renal replacement therapy is required, because renal recovery may occur after prolonged dialysis. A wrongly withdraws the disease-specific therapy in favour of labetalol. B overweights the microangiopathic features; plasma exchange would be appropriate for a clinical syndrome strongly suggesting immune TTP, but it does not replace ACE inhibition or urgent dialysis here. C delays dialysis despite life-threatening complications. E maintains renin–angiotensin blockade but substitutes an angiotensin receptor blocker, which does not have equivalent evidence to a tolerated ACE inhibitor in established scleroderma renal crisis.

Reference: The 2024 British Society for Rheumatology guideline for management of systemic sclerosis (2024) — https://pubmed.ncbi.nlm.nih.gov/39255973/ UK Scleroderma Study Group guidelines on the diagnosis and management of scleroderma renal crisis (2016) — https://pubmed.ncbi.nlm.nih.gov/27749244/ Acute kidney injury: prevention, detection and management — Recommendations (2019; updated 2024) — https://www.nice.org.uk/guidance/ng148/chapter/Recommendations