Scleroderma Renal Crisis: Primary Treatment
ACE inhibitors must be started immediately at diagnosis of scleroderma renal crisis and aggressively dose-escalated to control blood pressure—this intervention improves 1-year survival from 15% to 76%. 1, 2
Immediate Management
Start ACE inhibitors without delay upon diagnosis, regardless of blood pressure level. 1 The most extensively studied agents are captopril and enalapril, which should be titrated to maximum tolerated doses to achieve rapid blood pressure control. 2, 3
- Captopril is preferred initially due to its short half-life allowing rapid dose titration and easier management if hypotension occurs 3, 4
- Continue ACE inhibitors even if dialysis becomes necessary, as renal recovery can occur 3-18 months later 5
- The recommendation carries a strength of C from EULAR guidelines due to lack of randomized trials, but the survival benefit is so dramatic (66% at 5 years vs 10% without treatment) that RCTs are considered unethical 1, 2
Additional Antihypertensive Therapy
If blood pressure remains uncontrolled despite maximum ACE inhibitor doses, add calcium channel blockers as second-line agents, followed by diuretics and alpha-blockers in third line. 6 Do not delay ACE inhibitor initiation to try other agents first—ACE inhibitors specifically address the renin-angiotensin-mediated intrarenal vasoconstriction that drives scleroderma renal crisis. 2, 7
Critical Monitoring Requirements
All scleroderma patients receiving glucocorticoids require regular blood pressure and renal function monitoring to detect scleroderma renal crisis early. 1
- Patients on ≥15 mg/day prednisone have 4.4-fold increased risk of scleroderma renal crisis (OR 4.4; 95% CI 2.1-9.4) 2, 5
- High-dose steroids (≥30 mg/day) are particularly associated with normotensive scleroderma renal crisis, which carries worse prognosis 1, 2
- Highest risk patients include those with diffuse cutaneous disease in first 4-5 years, rapidly progressive skin thickening, or recent corticosteroid exposure 2
Dialysis Considerations
Approximately 50% of patients requiring dialysis can eventually discontinue it, particularly those achieving excellent blood pressure control with ACE inhibitors. 7, 8 Patients needing dialysis beyond 2 years should be evaluated for renal transplantation, which has 82% 5-year survival in this population. 6
Common Pitfalls
The evidence does not support prophylactic ACE inhibitor use to prevent scleroderma renal crisis in normotensive patients—this strategy has not been shown to decrease risk or improve outcomes. 1 ACE inhibitors are reserved for treatment of established crisis, not prevention.