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Aim: End-stage renal disease (ESRD) is a common finding in systemic lupus erythematosus (SLE) and may contribute to mortality. The purpose of the study was to investigate the incidence of ESRD and all-cause mortality and their risk factors in patients newly diagnosed with SLE in Taiwan. Methods: This nationwide cohort study used data from the National Health Insurance Research Database. We identified 4130 newly diagnosed SLE patients at risk for ESRD during 2000-2002; among them, 103 developed ESRD by the end of 2008. Additional 412 age- and sex-matched incident ESRD non-SLE patients served as controls for the survival analysis. Results: Of the newly diagnosed SLE patients, 2.5% developed ESRD. Age (adjusted hazards ratio [HR] 0.66 for each 1-year increase; 95% confidence interval [CI] 0.47-0.94) and male gender (adjusted HR 2.24; 95% CI 1.4- 3.6) were significantly associated with ESRD development. Survival analysis conducted after ESRD development revealed a higher mortality risk among the older patients (HR 1.04; 95% CI 1.02-1.05). Survival analysis in the younger population (age < 40 years) after ESRD development revealed a significant mortality risk among SLE patients (HR 2.73; 95% CI 1.22-6.07). Conclusion: In the follow-up of newly diagnosed SLE patients in Taiwan, younger age and male gender were risk factors for ESRD development. After entering ESRD, these risk factors had different impacts on mortality. Despite the overall improvement in care of patients with lupus nephritis, survival is still poorer in the younger age population. [ABSTRACT FROM AUTHOR]