Introduction The mortality rate of childhood-onset systemic lupus erythematosus (cSLE) remains high due to the severity of the disease and it complications. The cause of death varies widely depending on the major organs involved and therapy received.

Objective To evaluate the causes of death of cSLE.

Methods The medical records of children aged <18 years who had been diagnosed with SLE following American Rheumatism Association criteria from 1985 – 2016 in the Division of Nephrology, Department of Paediatrics, Prince of Songkla University, Thailand, were reviewed.

Results There were 331 patients, 272 girls and 59 boys, of whom 77 (23.3%) died, 28.6% within the first year after diagnosis. Only 29 medical records were available for evaluation of cause of death. Of these, there were 7 boys and 22 girls with a mean age at presentation of 11.0±3.0 years. The mean follow-up duration was 4.6±3.7 (range 0.2–12.6) years. The major cause of death was sepsis (13) followed by acute respiratory distress syndrome (ARDS) (6), severe heart condition (3), acute kidney injury (AKI) (2), chronic kidney disease (CKD) (2) and intracranial haemorrhage (1). Conditions at the time of death were sepsis (25), pneumonia (16), acute kidney injury (15), bleeding disorders (11), neurological complications (10), ARDS (10), CKD (4), AKI on top of CKD (3).

Conclusion The cause of death in cSLE is usually multi-factorial and it is difficult to assign a single dominant cause. Sepsis remains the most common cause of death. In the long-term, end-stage renal disease emerges as an important cause of death in RRT limited institutions.