Scleroderma Renal Crisis!

Thanks, Dr. Levene, for presenting a great case of scleroderma renal crisis for us this week at noon report!

A 50 year-old male with history of systemic sclerosis presented as a transfer from another hospital for management of acute renal failure in the setting of gastroenteritis. There was initially concern for pre-renal AKI secondary to GI losses and so his ACEI had been held. On transfer, however, he became increasingly hypertensive with worsened creatinine at arrival. Urine studies showed trace protein without casts. He had a new mild anemia without evidence of hemolysis. He was rapidly started on captopril for presumed scleroderma renal crisis. His hypertension and kidney function improved over the course of several days without a need for dialysis and he was discharged home.

Scleroderma…Systemic Sclerosis…CREST…Help me out here

Terminology gets confusing. Here’s a quick framework (and some clarification on terms).

Scleroderma = Systemic Sclerosis

CREST is a variant of Limited (lcSSc)

Slide courtesy of Dr. Julianna Desmarais of rheumatology. “Scleroderma and Mixed Connective Tissue Disease”. Noon conference. 6/4/2019

Slide courtesy of Dr. Julianna Desmarais of rheumatology. “Scleroderma and Mixed Connective Tissue Disease”. Noon conference. 6/4/2019

Scleroderma Renal Crisis (SRC)…Heard of it but remind me…

 A medical emergency! Serious manifestation of Systemic Sclerosis characterized by:

Abrupt onset of moderate to severe hypertension

  • >140/90 or an increase above baseline of >30 SBP or >20 DBP

AND at least one of the following:

  • Acute kidney injury

  • Urinalysis that is normal or reveals only mild proteinuria with few cells or casts

  • ·Evidence of microangiopathic hemolytic anemia 

Risk Factors:

  • Early in the course of disease (usually first 3-5 years)

  • Rapidly progressive skin disease or diffuse disease

  • Tendon friction rubs

  • Recent corticosteroid use

  •   RNA polymerase III antibody positivity


  • Immediate reduction of blood pressure WITH AN ACE INHIBITOR (typically captopril)

    • Prospective cohort study of 108 patients with SRC showed 5-year survival of 65% vs. 10% (p<0.001) for those treated with ACE inhibitors vs. no ACE inhibitors respectively  (Steen et al)

  •   Continue an ACE inhibitor on discharge

  • Interestingly (and unfortunately) data suggests that ACE inhibitors are not effective as prophylaxis against development of SRC


DynaMed [Internet]. Ipswich (MA): EBSCO Information Services. 1995 - . Record No. T1555509169655Kidney Disease in Systemic Sclerosis; [updated 2018 Apr 12, cited place cited date here]. Available from Registration and login required.

Steen VD, Costantino JP, Shapiro AP, Medsger TA. “Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors.” Ann Intern Med. 1990 Sep 1;113(5)352-7