Rheumatology Question 12 Instructor

What class of medication can be life-saving in scleroderma renal crisis?

Scleroderma renal crisis is a syndrome that occurs primarily in patients with diffuse systemic sclerosis, usually within the first few years of the disease. The use of systemic steroids is thought to be a trigger for scleroderma renal cisis, which is characterized by a rise in blood pressure accompanied by creatinine rise and microangiopathic hemolytic anemia.

Prior to the advent of ACE-inhibitors, the one year survival of this renin-mediated phenomenon was 15%, while renal survival was virtually nil. With the use of ACE-inhibitors, the survival rates are closer to 76%, and the kidneys remain functional in 55% of patients. ARBs do not seem to have the same effects as ACE-Inhibitors in scleroderma renal crisis.

Steen VD, Costantino JP, Shapiro AP. 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

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