Scleroderma Renal Crisis

This patient has several features of Scleroderma (=Systemic sclerosis). You may note the tightness of the skin around the face and hands. Scleroderma renal crisis is characterized by systemic hypertension, marked elevation of plasma renin, and a rapid deterioration in renal function. Often there is an associated microangiopathic hemolytic anemia (1). Renal crisis reportedly occurs in about 10-45% of patients with scleroderma within 4 years of presentation (2,3). There was initially a fear that this was induced by glucosteroids but most cases occur in the absence of preceeding steroid therapy (4). However, in a few patients who develop acute renal failure without hypertension there had been an increase in steroid use just prior (3). Prior to hemodialysis, there was a 100% rate of mortality within one year of developing this syndrome. With dialysis this rate had dropped to 85% and with ACE inibitors it has fallen to 27% (5). Predictors of poor outcome include male sex, increasing age, poor left ventricular function and a serum creatinine greater than 3.0 mg/dL. Of note, there is one published case report of Cyclosporin helping in 3 out 8 patients which needs further evaluation (6).

References:

  1. Collins DA, Patel S, Eastwood JB, Bourke BE. Favourable outcome of scleroderma renal crisis. J R Soc of Med 1996; 89: 49P-50P.
  2. Cannon PJ, Hassar M, Case DB, Casarella WJ, Sommers SC, Leroy EC. The relationship of hypertension and renal failure in scleroderma (progressive systemic sclerosis) to structural and functional abnormalities of the renal cortical circulation. Medicine (Baltimore) 1974; 53:1-46.
  3. Helfrich DJ, Banner B, Steen VD, Medsger TA. Normotensive renal failure in systemic sclerosis. Arthritis Rheum 1989; 32:1128-34.
  4. Steen VD, Medsger TA, Osail TA, Zeigler GL, Shapiro AP, Rodnan GP. Factors predicting development of renal involvement in progressive systemic sclerosis. Am J Med 1984; 76:779-86.
  5. Steen VD, Constantino 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; 113:354-7.
  6. Denton CP, Seny P, Abdulla A, Black CM. Case Reports: Acute renal failure occurring in scleroderma treated with cyclosporin A: A report of three cases. Br J Rheum 1994; 33:90-92.

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