scleroderma renal crisis

Etiology

• almost exclusively with early cutaneous scleroderma
• risk factors:
  • glucocorticoid use
  • pericardial effusion

Epidemiology

• 10-15% of patients with systemic sclerosis
  • more frequent in patients with scleroderma than limited cutaneous sclerosis
• 80% female

Pathology

• intimal proliferation & luminal thrombosis in the afferent renal arterioles
• thrombotic microangiopathy with glomerular ischemia
• hyperreninemia

Clinical manifestations

• hypertension often severe
  • some patients may be normotensive (10%)
• renal failure
• microangiopathic hemolytic anemia
• variable features
  • pulmonary edema
  • hypertensive encephlopathy

Laboratory

• complete blood count may show anemia, thrombocytopenia
• peripheral blood smear may show microangiopathic hemolytic anemia
• renal function tests consistent with acute renal failure
• plasma renin is high
• RNA polymerase 3 Ab in serum

Management

• a medical emergency
• hospitalization for aggressive blood pressure control
• ACE inhibitors are standard therapy even in the face of renal failure (including dialysis) regardless of blood pressure
  • captopril is the preferred ACE inhibitor
    • most extensively studied
    • short 1/2 life allows rapid titration
• hemodialysis as needed
  • 65% of patients require hemodialysis
  • renal function may improve months after initiation of dialysis
• prognosis
  • 5 year mortality 40%, higher in men

More general terms

• scleroderma (diffuse cutaneous systemic sclerosis)
• acute renal failure (ARF)