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

Abstract

BackgroundMorbidity 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.MethodsWe 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.ResultsThere 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).ConclusionIn 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.

Childhood-Onset Systemic Lupus Erythematosus: Southeast Asian Perspectives

“…However, a more recent study showed marked improvement in survival with the country’s economic development and accessibility to healthcare with a mortality of only 1.6% in a 5-year cohort on par with other developed countries in the world [ 29 , 47 , 48 ]. Mortality rates in Thailand (23.3–31.2%) remain high despite improvement over the years with most deaths occurring within the first year of presentation [ 49 , 50 ]. Dung et al reported similar findings of high incidence of early deaths in 8.9% of their cohort in Vietnam within the first six months of diagnosis [ 19 ].…”

“…Dung et al reported similar findings of high incidence of early deaths in 8.9% of their cohort in Vietnam within the first six months of diagnosis [ 19 ]. These could reflect the high prevalence of renal disease in these countries with poor healthcare accessibility, delayed diagnosis, and limited paediatric rheumatology expertise [ 45 , 49 ]. Meanwhile, the mortality rates of 7.6% (5-year follow-up) in the Philippines and 5.7% (10 year follow up) in Malaysia are comparable to other developed countries [ 21 , 24 ].…”

Improving musculoskeletal health for children and young people – A ‘call to action’

“…There is also a markedly increased risk of cardiovascular disease in adulthood [ 84 , 85 ]. Ten-year survival for JSLE in LRIC and MRIC is approximately 80% [ 2 , 80 , [86] , [87] , [88] , [89] , [90] ] compared with 99% in HRIC [ 85 ], with lupus nephritis and infection, the major causes of death [ 91 , 92 ].…”

Epidemiology and risk factors for avascular necrosis in childhood systemic lupus erythematosus in a Taiwanese population

“…Despite a general lack of comorbid conditions, previous studies have shown that patients with childhood SLE are at a greater risk of mortality compared to patients with adult SLE, and especially those with LN 12,13 . With earlier recognition of the disease, increased availability of new immunosuppressants and improved intensive care, the 5-year and 10-year survival rates for childhood SLE have improved considerably in the last decade 14 . As survival rates for childhood SLE have improved, prevention and early treatment of morbidities resulting from disease activity and adverse effects of treatment, and especially corticosteroids and immunosuppressants, have become increasingly important.…”