A 42-year-old woman with a 3-year history of diffuse cutaneous systemic sclerosis comes to the emergency department due to a severe headache and blurred vision that began 24 hours ago. Her temperature is 37.0°C (98.6°F), blood pressure is 218/116 mm Hg, and pulse is 92/min. Physical examination shows tight, thickened skin over the face, trunk, and extremities. Laboratory studies show a serum creatinine of 2.9 mg/dL (baseline 0.9 mg/dL) and a hemoglobin of 10.2 g/dL. A peripheral blood smear reveals numerous schistocytes. Which of the following is the most appropriate next step in the management of this patient?
- Administration of captoprilAnswer
- BAdministration of labetalol
- CAdministration of methylprednisolone
- DInitiation of hemodialysis
- ERenal ultrasonography
Answer
Administration of captopril
This patient's presentation of acute malignant hypertension, acute kidney injury, and microangiopathic hemolytic anemia in the setting of diffuse cutaneous systemic sclerosis is the classic description of Scleroderma Renal Crisis (SRC). The underlying pathophysiology involves thickening of the renal arcuate and interlobular arteries, leading to decreased renal perfusion, massive renin release, and subsequent severe hypertension. The cornerstone of life-saving therapy is the prompt administration of an angiotensin-converting enzyme (ACE) inhibitor, classically captopril. ACE inhibitors directly block the maladaptive renin-angiotensin-aldosterone system cascade, relieving renal vasoconstriction.
Step-by-Step Solution
Key Concept
Scleroderma Renal Crisis (SRC) presents with malignant hypertension and oliguric acute renal failure in a patient with systemic sclerosis; the primary treatment is immediate initiation of an ACE inhibitor.
Estimated Time:45s