Scleroderma Renal Crisis

Take Home Points: Scleroderma Renal Crisis

What is SRC? – a sudden onset of malignant hypertension and renal failure that occurs in a minority of scleroderma patients.

Pathogenesis – thought to involve small interlobular arteries in the kidneys which develop intimal proliferation and spasm à vasculopathy that reduces renal blood flow à activation or RAAS à hypertension and worsened renal dysfunction

Epidemiology and Onset of Disease

  • Occurs in 5-20% of pts with diffuse cutaneous SSc, much less in pts with limited cutaneous SSc
  • Occurs early in the disease – almost all in first 5 years of diagnosis of SSc, median time of 7.5 months from first non-Raynaud symptom of SSc!
  • Possible risk factors include – use of high dose glucocorticoids, SSc with diffuse skin involvement, ? anti- RNA polymerase III antibody

3 Major Features of SRC

  • Abrupt onset mod-severe HTN associated with increased plasma renin activity
    • About 10% of pts will NOT have hypertension!
  • Acute kidney injury
  • Essentially normal urinalysis with only mild proteinuria and few cells or casts
  • Other associated features: microangiopathic hemolytic anemia, CHF, pulmonary edema, hypertensive retinopathy and encephalopathy and seizure

Treatment of SRC – untreated, can progress to ESRD in months & death within a year

  • ACE INHIBITORS!! Key is to initiate before the onset of permanent renal damage.
  • Most evidence with captopril, which is short acting and can be titrated quickly
  • Goal is to return pt to baseline BP within 72hrs of starting treatment
  • Low dose ACE-I should be continued indefinitely even if BP returns to normal


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