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)
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
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. T1555509169655, Kidney Disease in Systemic Sclerosis; [updated 2018 Apr 12, cited place cited date here]. Available from https://www.dynamed.com/topics/dmp~AN~T1555509169655. 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