Scleroderma renal crisis

Clinical Manifestations

• Acute onset renal failure without another cause
  • Bland urine sediment, though can have proteinuria and hematoria
• Acute onset moderate tosevere hypertension, often with hypertensive emergency
  • Papilledema
  • Hypertensive retinopathy
  • Hypertensive encephalopathy
• Additional findings
  • Bicytopenia with MAHA and thrombocytopenia
  • Flash pulmonary edema
  • New-onset proteinuria or hematuria without other cause

Management

• Blood pressure control, primarily with ACE inhibitors, is the primary treatment
  • First-line: captopril has the most evidence
    • Initial dose 6.25 to 12.5 mg, increase by 12.5 to 25 mg every 4 to 8 hours to achieve target
  • Alternatives: enalapril or ramipril
  • Target return to baseline BP within 72 hours, with about 20 mmHg reduction daily
• For CNS disease, can add nitroprusside
• Monitor creatinine

Prevention

• No clear role for ACE inhibitors for primary prevention
• ACE inhibitors are continued indefinitely for secondary prevention
• Avoid beta blockers, which can worsen Raynaud phenomenon
• Avoid glucocorticoids if possible, as they can precipitate crises

Prognosis

• Untreated, SRC progresses to ESRD within month
• Treated, 20 to 50% still end up on dialysis