A 45-year-old woman with systemic sclerosis (diffuse cutaneous SSc) develops acute onset hypertension (220/130 mmHg), rising creatinine, microangiopathic hemolytic anemia, and thrombocytopenia. Which treatment is most critical to preserve renal function?
- A IV methylprednisolone
- B ACE inhibitor (captopril) ✓
- C Plasma exchange (plasmapheresis)
- D Cyclophosphamide IV pulse
Explanation
Scleroderma renal crisis (SRC) is the most feared acute complication of diffuse cutaneous systemic sclerosis, typically occurring within the first 4 years of disease onset. It is characterized by accelerated hypertension, oliguric AKI, MAHA, and thrombocytopenia. ACE inhibitors (captopril is preferred for its rapid action) are the cornerstone of treatment and have dramatically improved outcomes — they work by blocking the renin-angiotensin system activation central to SRC pathogenesis. Steroids are associated with precipitating SRC. Plasmapheresis has no proven benefit.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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