Scleroderma Renal Crisis: Symptoms and Treatment
Clinical Presentation
Scleroderma renal crisis (SRC) presents with malignant hypertension (blood pressure >150/85 mmHg or ≥20 mmHg increase from baseline systolic), acute kidney injury (serum creatinine increase ≥10%), and often severe headache, with or without oliguria, though a normotensive variant exists and carries worse prognosis. 1, 2
Key Symptoms
- Severe headache associated with nausea and vomiting from hypertensive emergency 3
- Acute hypertension (>150/85 mmHg) in approximately 84% of cases, though 16% present with normotensive SRC 4, 2
- Oliguria or anuria indicating acute renal failure 4
- Left ventricular heart failure and hypertensive encephalopathy as typical features 4
- New-onset anemia from thrombotic microangiopathy, present in approximately 43-50% of cases 4, 5
High-Risk Clinical Context
SRC typically occurs in patients with:
- Diffuse cutaneous systemic sclerosis within the first 3-5 years of disease, particularly with rapidly progressive skin thickening 5, 2
- Recent glucocorticoid exposure, especially ≥15 mg/day prednisone (4.4-fold increased risk; OR 4.4; 95% CI 2.1-9.4) 6, 7
- Palpable tendon friction rubs and new cardiac events 1
Treatment Approach
Immediate initiation of high-dose ACE inhibitors is the cornerstone of treatment and dramatically improves survival from 15% to 76% at 1 year. 8, 6
Acute Management Algorithm
Start ACE inhibitors immediately upon diagnosis with aggressive dose escalation to control blood pressure 8, 6
Add additional antihypertensive agents as needed for rapid blood pressure control if ACE inhibitors alone are insufficient 5, 4
Initiate dialysis when indicated, but recognize that approximately 50% of patients can eventually discontinue dialysis with optimal blood pressure control 4
Continue ACE inhibitors indefinitely as long as there is any chance for additional improvement in kidney function 7
Critical Treatment Caveat
In pregnant patients with active SRC, ACE inhibitors should still be used despite their teratogenic potential, because the risk of maternal or fetal death from untreated SRC exceeds the medication risk. 7 This represents one of the rare circumstances where ACE inhibitors are justified during pregnancy.
Evidence Does NOT Support Prophylactic Use
Published evidence does not support preventive use of ACE inhibitors to decrease risk of development or improve outcome of SRC in asymptomatic patients. 7 Only two patients in one cohort were taking small doses of ACE inhibitors prior to SRC onset, and prophylactic use remains unproven 9
Prognosis with Treatment
Survival rates with ACE inhibitor treatment are:
- 76% at 1 year and 66% at 5 years (compared to 15% at 1 year and 10% at 5 years without ACE inhibitors) 7, 6
- More recent studies show 71-82% survival at 1 year, 59-60% at 5 years, and 42-47% at 10 years 7
- Normotensive SRC carries worse prognosis and is particularly associated with high-dose steroids (≥30 mg/day prednisone) 7, 6
Monitoring Requirements for At-Risk Patients
Blood pressure and renal function should be carefully monitored in all systemic sclerosis patients treated with glucocorticoids, particularly those on ≥15 mg/day prednisone. 7, 6
Patients requiring close surveillance include those with:
- Diffuse cutaneous systemic sclerosis in first 4-5 years of disease 6, 2
- Rapidly progressive skin thickening 2
- Recent corticosteroid exposure, especially ≥15 mg/day 6
- High skin scores and joint contractures 7
Common Pitfall to Avoid
High-dose glucocorticoids (≥30 mg/day) are strongly associated with normotensive SRC, which can be easily confused with other causes of acute kidney injury and carries worse prognosis. 7, 6, 2 Maintain high clinical suspicion for SRC even in the absence of hypertension when patients on high-dose steroids develop acute renal dysfunction.