Introduction Systemic lupus erythematosus (SLE), one of the most common connective tissue diseases in children, causes high morbidity and mortality. Childhood onset SLE is more severe than adult onset SLE. Advances in medical technology and management have improved SLE outcomes; however there has been no recent study to investigate long-term outcomes, including gender-specific ones, in a large single group of children.

Objective To describe the mortality of childhood SLE in a single tertiary care centre in southern Thailand over a 30 year period, compare trends in survival over time and determine predictors for survival.

Methods We retrospectively reviewed the medical records of children (aged<15 years) who were diagnosed with SLE at the Department of Paediatrics, Songklanagarind Hospital, from 1985–2014 and were followed up for at least 2 years. Gender-specific mortality and survival rates were compared across three decades, and survival rates compared for different LN classifications.

Results The study included 322 children (263 girls, 59 boys) having a mean age at presentation of 11.5±2.6 years. During a mean follow-up of 6.9±5.1 (range 2–28) years, 77 children (23.9%) died, 28.6% within the first year. Renal biopsy results in 249 children (77.3%) showed LN classes I, II, III, IV, and V in 10, 68, 11, 138, and 20 children, respectively.

The overall mortality rate was 3.5 per 100 person years. Survival rates at 1, 5 and 10 years were 93.1%, 82.4% and 71.6%, respectively. Ten-year survival rates for children diagnosed in the decades 1985–1994, 1995–2004 and 2005–2014 were 67.4%, 63.2% and 80.6%, respectively (p<0.001).

Boys had worse survival than girls (hazard ratio=2.3, 95% CI: 1.4–3.7) even after adjusting for decade of diagnosis. There was no difference in survival among the LN classes (p=0.99).

Conclusion In our setting, the survival rate of childhood onset SLE has improved over the past 10 years, but mortality is still high compared to developed countries, particularly in boys. Deaths occurred within the first year of presentation more than in subsequent years. Severity of lupus nephritis did not determine survival.