title: Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors.
abstract: OBJECTIVE
  To determine the outcome of scleroderma renal crisis before and after the availability of angiotensin converting enzyme (ACE) inhibitors.
  
  
  METHODS
  Evaluation of a large cohort of patients with systemic sclerosis and renal crisis who were followed prospectively.
  
  
  METHODS
  University scleroderma center.
  
  
  METHODS
  One hundred and eight patients who had scleroderma renal crisis between 1972 and 1987.
  
  
  METHODS
  ACE inhibitors.
  
  
  RESULTS
  Therapy with ACE inhibitors has dramatically improved the survival of patients with scleroderma renal crisis (1-year survival, 15% without and 76% with ACE inhibitors; P less than 0.001). However, 24 (44%) of 55 patients with scleroderma renal crisis who were treated with ACE inhibitors died early or required permanent dialysis. Older age, male sex, an initial serum creatinine level of more than 270 mumol/L, inadequately controlled blood pressure, and congestive heart failure were associated with these poor outcomes, but only older age and congestive heart failure were significant in a multivariate logistic regression analysis. Eleven of twenty patients (55%) who survived dialysis more than 3 months and continued to receive therapy with ACE inhibitors were able to discontinue dialysis after 3 to 15 months compared with 0 of 15 dialysis patients who did not receive ACE-inhibitor therapy (P = 0.002).
  
  
  CONCLUSIONS
  Patients with systemic sclerosis who develop hypertension should be treated with an ACE inhibitor. Improved survival and successful discontinuation of dialysis are possible when ACE inhibitors are used to treat scleroderma renal crisis.
authors.affiliation: University of Pittsburgh School of Medicine, Pennsylvania.
authors.forename: V D
authors.initials: VD
authors.lastname: Steen
authors.forename: J P
authors.initials: JP
authors.lastname: Costantino
authors.forename: A P
authors.initials: AP
authors.lastname: Shapiro
authors.forename: T A
authors.initials: TA
authors.lastname: Medsger
mesh_terms: D058186:Acute Kidney Injury / Q000188:drug therapy* / Q000209:etiology / Q000401:mortality / Q000628:therapy; D000368:Aged; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D006973:Hypertension / Q000188:drug therapy* / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010414:Pennsylvania; D011446:Prospective Studies; D012044:Regression Analysis; D006435:Renal Dialysis; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000150:complications*; D015996:Survival Rate
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors