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Subjects/Dermatology/Scleroderma
Scleroderma
hard
hand Dermatology

A 38-year-old female with a known history of diffuse cutaneous systemic sclerosis presents to the emergency department with sudden onset severe headache, blurred vision, and blood pressure of 200/120 mmHg. Laboratory tests reveal acute kidney injury with a serum creatinine of 3.5 mg/dL (baseline 0.9 mg/dL) and microangiopathic hemolytic anemia. Which of the following is the most appropriate initial management step?

A. A. Intravenous corticosteroids
B. B. Oral cyclophosphamide
C. C. Intravenous ACE inhibitor (e.g., enalaprilat)
D. D. Plasmapheresis

Explanation

The patient's presentation with sudden onset severe hypertension, acute kidney injury, and microangiopathic hemolytic anemia in the context of diffuse cutaneous systemic sclerosis is highly suggestive of Scleroderma Renal Crisis (SRC). SRC is a medical emergency requiring prompt treatment. The cornerstone of treatment for SRC is the rapid administration of ACE inhibitors, which have been shown to significantly improve renal outcomes and survival. Intravenous ACE inhibitors are preferred in severe cases. Corticosteroids are generally contraindicated in SRC as they can worsen the condition. Cyclophosphamide is used for interstitial lung disease, not SRC. Plasmapheresis is not the primary treatment for SRC.

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