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Background Systemic lupus erythematosus (SLE) is a devastating systemic disease that can lead to end-stage renal disease (ESRD). Our goal was to assess the relative mortality risk associated with race in pediatric and adult populations with ESRD secondary to SLE maintained on hemodialysis (HD). Methods We identified an inception cohort of patients who were started on HD in January 1990 from data collected by the United States Renal Data System (USRDS). Kaplan–Meier survival analyses were performed in these patients using the time at risk from 1 January 1990 through 31 December 2010, the last date of the USRDS data collection period in this dataset. Cox proportional hazard models were used to assess mortality, adjusted for age at dialysis initiation. Subjects were censored at transplantation or end of follow-up. Results There were 1580 patients with ESRD secondary to SLE, 252 pediatric patients (62% African American) and 1328 adults (56% African American). African American pediatric patients with ESRD secondary to SLE had a 2-fold increased risk of death compared with African American children with other causes of ESRD [hazard ratio (HR): 2.1, 95% confidence interval (CI): 1.4–2.9, P < 0.01]. Increased risk of death was also seen in African American adults with ESRD secondary to SLE compared with both Caucasians with ESRD secondary to SLE (HR: 2.3, 95% CI: 1.2–4.2, P < 0.01) and African American adults with ESRD secondary to other diseases (HR: 1.2, 95% CI: 1.1–1.4, P < 0.01). Conclusion Our study suggests that there is a significant increased risk for mortality in African American children and adults with ESRD secondary to SLE. This suggests that African Americans with ESRD secondary to SLE need aggressive monitoring. [ABSTRACT FROM PUBLISHER]