Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

Abstract

Abstract Background Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to describe the mortality of childhood SLE in a single tertiary care centre over three decades, compare trends in survival over time, and determine predictors for survival. Methods We retrospectively reviewed the medical records of children aged < 18 years who were diagnosed with SLE at the Department of Pediatrics, Songklanagarind Hospital, Thailand, from 1985 to 2016. Results There were 331 children (272 girls, 59 boys) with a mean age at presentation of 11.5 ± 2.6 years. The mean follow-up duration was 7.0 ± 5.0 (range 1–28) years, 77 children (23.3%) died, 28.6% within the first year after diagnosis. The overall mortality rate was 3.3 per 100 person-years. Survival rates at 1, 5 and 10 years were 93.4%, 83.1% and 72.6%, respectively. Ten-year survival rates for the children diagnosed in the decades 1985–1996, 1997–2006 and 2007–2016 were 67.4%, 63.4% and 82.8%, 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. Lupus nephritis (LN) class IV had similar survival compared to LN classes II/III/V combined (hazard ratio = 1.0, 95% CI: 0.6–1.7). Conclusion In our setting, the survival rate of childhood onset SLE has improved during the past 10 years, but mortality is still high compared to developed countries, particularly in boys.

Keywords

• Childhood-onset
• Survival rates
• Systemic lupus erythematosus
• Thai