A 58-year-old woman with diffuse cutaneous systemic sclerosis for 6 years presents with acute-onset severe headache, blurred vision, and blood pressure 210/130 mmHg. Fundoscopy shows papilledema and retinal hemorrhages. Creatinine has risen from 1.0 to 3.2 mg/dL in 2 weeks. Peripheral blood smear shows schistocytes. Platelet count is 92,000/µL. What is the most appropriate immediate treatment?

• A Intravenous nitroprusside for blood pressure control
• B Plasma exchange
• C Angiotensin-converting enzyme inhibitor (captopril) ✓
• D High-dose intravenous methylprednisolone

Explanation

This is scleroderma renal crisis, characterized by malignant hypertension, thrombotic microangiopathic hemolytic anemia (schistocytes, thrombocytopenia), and renal failure in diffuse SSc. ACE inhibitors (captopril) are the specific treatment and have dramatically improved survival. Nitroprusside or other agents may be used adjunctively but ACE inhibition is essential. Plasma exchange is reserved for TTP (ADAMTS13 deficiency). Corticosteroids increase risk of SRC and are contraindicated.

Reference: Kelley and Firestein's Textbook of Rheumatology, 11th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.