Scleroderma Renal Crisis

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Scleroderma Renal Crisis


  • abrupt onset of accelerated hypertension
  • renal impairment
  • life-threatening
  • patient awareness critical
  • outcome transformed by ACE inhibitors
  • affects 10% of diffuse cutaneous and 1% of limited cutaneous

Management

  • ACE-inhibitors
  • iloprost
  • gradual blood pressure lowering (unless compelling reason for lowering)
  • renal biopsy when safe
  • HDU nursing
  • renal support
  • plasma exchange
 

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