UNLABELLED: BACKGROUND: Incidence and disease pattern of childhood-onset SLE is reported to differ among ethnic groups. METHODS: To describe disease pattern and 6 month follow-up in a referral based cohort of 45 Vietnamese children with SLE. Forty-five children who were subsequently diagnosed to have systemic lupus erythematosus (f/m = 4/1) were referred to the Ho Chi Minh City Children's Hospital No.1 during a 12-month period in 2009. RESULTS: The mean age at diagnosis was 12.8 years (SD = 2.5). Thirty-seven (82%) fulfilled criteria for lupus nephritis (LN). At diagnosis, impressively high SLEDAI and ECLAM scores were recorded (mean and SD), 23.8 (11.6) and 6 (2.3), respectively. The mean renal SLEDAI score was 8.2. The mean haemoglobin (g/dL, SD) was 8.5 (2.1). The Coombs test was positive in 30 of 36 children (83%). The mean plasma creatinine was 0.98 (SD 1.2) and mean Westergren sedimentation rate was 83.6 (SD 37.4). The patient age at diagnosis was positively correlated to the SLEDAI (p = 0.034) and ECLAM (p = 0.022). At 6 month follow-up of the 45 children, 15 patients were in complete remission, 5 were in partial remission, 6 had stable disease, 3 had relapsed, 3 had evolving disease, 2 had ongoing resistant disease and 4 had died. Seven patients were lost to follow-up. A second renal biopsy showed an improved ISN class in 13 of 15; in 2 cases the ISN class remained unchanged. CONCLUSIONS: Forty-five Vietnamese children with SLE were referred to Ho Chi Minh Children's Hospital No. 1 during a16 month period from 2008-2009. These patients had a strikingly high prevalence of Coombs positive anaemia, a high prevalence of lupus nephritis, and very high SLEDAI and ECLAM scores at the time of diagnosis. While there may be referral biases, our Vietnamese SLE patients appear to have severe disease upon presentation but do reasonably well in the short-term.