Scleroderma renal crisis.

Abstract

Abrupt onset of severe uncontrolled hypertension and rapidly progressive oliguric renal failure characterizes scleroderma renal crisis. The etiology is unclear, but very high renin levels are present. While scleroderma is more common in women and whites, there is no difference in the prevalence of scleroderma renal crisis by gender. However, there appears to be a higher prevalence of scleroderma renal crisis among African Americans than whites. Survival was dismal prior to the introduction of the vigorous treatment of hypertension and use of converting-enzyme inhibitors. However, most data on the benefit of these medications are derived from uncontrolled and unblinded studies. Prospective, randomized controlled trials are needed to assess the role of angiotensin receptor blockers. Prevention trials could define the role of various drugs in decreasing the rate of scleroderma renal crisis.