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.

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Background Previous studies have shown that direct oral provocation tests, without prior skin testing, in children having delayed onset, benign rashes to beta-lactam antibiotic is safe and effective. Although, this test is useful in confirming drug hypersensitivity reactions, there is no standard protocol recommendation of drug provocation tests. This study aimed to evaluate the safety of the direct oral provocation test, using the Amoxicillin-2-step-challenge without prior skin testing, in children with history of non-immediate reactions to amoxicillin. Methods The Amoxicillin-2-step-challenge protocol was performed in children with history of non-immediate reactions to amoxicillin. This protocol is composed of 2 doses of amoxicillin, with a 30-min interval; continued for a total of 5 days. All of the patients had not undergone skin testing before the oral provocation test. Results This study included 54 children, having a median age of 6.6 years, with 70.4% being male. Amoxicillin and amoxicillin-clavulanic acid were reported as the culprit drug in 75.9% and 24.1%, respectively. The index reactions were maculopapular (MP) rash in 79.6% and delayed urticarial rash/angioedema in 20.4%. Five patients (9.3%) had a reaction during the provocation test, all of these patients had delayed urticaria and were treated with oral antihistamine. However, 1 patient developed a fever alongside an MP rash. Laboratory investigation for this patient showed increased atypical lymphocytes and liver enzymes elevation. Conclusions Direct oral provocation tests, using the Amoxicillin-2-step-challenge, without prior skin testing, revealed good, immediate safety for the diagnosis of amoxicillin hypersensitivity in children with history of non-immediate reactions to amoxicillin.

IntroductionSystemic 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.ObjectiveTo 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.MethodsWe 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.ResultsThe 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).ConclusionIn 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.