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Systemic sclerosis renal crisis — ABIM Board MCQ

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HardRheumatology/Allergy/ImmunologySystemic sclerosis renal crisisABIM Board

A 49-year-old with diffuse cutaneous systemic sclerosis recently received high-dose prednisone and now has abrupt severe hypertension, microangiopathic hemolytic anemia and rapidly rising creatinine. What treatment should begin immediately?

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Correct answer: E — Start ACE inhibition with rapid dose titration

The best answer is “Start ACE inhibition with rapid dose titration”. This is scleroderma renal crisis, and immediate ACE inhibition is the treatment that transformed its prognosis. Creatinine may continue to rise initially and is not a reason to withhold the drug. High-dose glucocorticoids are a risk factor rather than treatment, and the systemic-sclerosis context distinguishes the syndrome from primary thrombotic thrombocytopenic purpura. Other antihypertensives may supplement but not replace ACE inhibition.

Reference: EULAR 2023 Systemic Sclerosis Treatment Recommendations: https://pubmed.ncbi.nlm.nih.gov/39874231/