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Poor prognostic signs of scleroderma renal crisis
How is scleroderma renal crisis treated?
Patients with SRC should be hospitalized. They should be put on a short-acting ACE inhibitor with the goal of decreasing systolic blood pressure by 20 mmHg within the first 24 hours. Hypotension should be avoided. The ACE inhibitor should then be maximized to normalize the blood pressure. Up to 30% of patients will have continued blood pressure elevation on ACE inhibitors. These patients should have calcium channel blockers added as second-line therapy, followed by angiotensin-receptor blockers. Endothelin receptor antagonists and prostacyclins have also been tried. Poor prognostic factors include: (1) male gender, (2) initial creatinine > 3mg/dL, (3) normotensive at onset, and (4) cardiac involvement with myocarditis or arrhythmias. Approximately 50% to 60% patients will require dialysis within the first 24 months, with half of those recovering enough renal function to stop dialysis. Therefore, ACE inhibitor therapy should be continued indefinitely even in patients who have progressed to dialysis, and kidney transplantation should be delayed at least 24 months.
