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Scleroderma renal crisis — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologyScleroderma renal crisisABIM Board

A 45-year-old with rapidly progressive diffuse systemic sclerosis develops BP 202/118 mm Hg, oliguria, creatinine 3.0 mg/dL, schistocytes and thrombocytopenia after receiving prednisone 40 mg/day. Which treatment should be started immediately?

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Correct answer: B — Begin captopril and titrate the dose against blood pressure

The best answer is “Begin captopril and titrate the dose against blood pressure”. The phenotype is scleroderma renal crisis with secondary microangiopathy; prompt ACE inhibition is lifesaving and should continue despite a possible early creatinine rise. Glucocorticoid exposure is a risk factor, and plasma exchange does not replace ACE inhibition unless a separate diagnosis is established.

Reference: American College of Rheumatology: Scleroderma Renal Crisis: https://rheumatology.org/patients/scleroderma