A total of 153 pediatric systemic lupus erythematosus patients (131 girls and 22 boys) under 18 years of age who were newly diagnosed between 1980 and 2001 were retrospectively analyzed. All patients were followed up until death, loss of follow-up, or December 31, 2002. The mean follow-up duration was 6.11 +/- 9.02 years. There were no differences in sex and family history between the 1980-to-1990 cohort and the 1991-to-2001 cohort. The rate of complications of infection, skeletal manifestation, and end-stage renal disease were significantly higher in the 1980-to-1990 cohort (p=0.007, 0.001, and 0.000, respectively). Infection was the leading cause of death, and there were no differences in the cause of death between the 2 cohorts except end-stage renal disease (p=0.005). The survival rate was significantly worse in the 1980-to-1990 cohort than in the 1991-to-2001 cohort (p=0.0012). The 2 significant poor prognostic factors were being female and the development of end-stage renal disease. We conclude that the mortality and morbidity of SLE has improved over the past 2 decades. It may be associated with newer treatment modalities and better supportive care available recently.