Of all patients with systemic lupus erythematosus (SLE), 15-20% are diagnosed during childhood, with disease onset prior to the age of 16 years. Because disease expression in SLE is influenced by environment factors and differs between racial and ethnic groups. The aims of this review were to describe prevalence, clinical manifestations, common infectious complications, and outcome of pediatric-onset SLE in Asia. The prevalence of pediatric-onset SLE was 6.3-19.3 per 100,000 in Asia. The ratio of female to male was 4.7-6.2. The mean age at diagnosis of pediatric-onset SLE was 8.6-13.5 years. The most common clinical features of pediatric-onset SLE in Asia were cutaneous rashes, arthritis, hematological involvement and nephritis. The occurrence of nephritis varies from 29% to 81%. The most common histopathology of lupus nephritis was diffuse proliferative glomerulonephritis (WHO Class-IV) which occurred in 39.4-54% of case of lupus nephritis. Pediatric-onset SLE patients with infections have poor outcomes than uninfected patients. Gram-negative bacilli are the most common microorganisms responsible for bacteremia in Asian patients with SLE. Recurrent major infections predict poorer disease outcome and associated organ damage in pediatric-onset SLE. Improving the survival of SLE patients was reported in Asia in recent decades. The survival was 92% at the age of 5 years, 86% at 10 years and 79% at 15 years in children with SLE in Taiwan in 2008.

This study aimed to compare differences of acute pancreatitis between adult- and pediatric-onset systemic lupus erythematosus (SLE) patients and to clarify the risk factors for mortality. Medical records that carried the dual diagnosis of SLE and acute pancreatitis between 1991 and 2005 were reviewed. Forty-eight episodes of acute pancreatitis were identified in 13 pediatric-onset SLE (pSLE) and 27 adult-onset SLE (aSLE) patients. The prevalence was 1.34% overall, with higher prevalence in pSLE (5.22%) compared with aSLE (0.99%) (p < 0.001). The SLEDAI score on presentation of acute pancreatitis was higher in pSLE (mean ± SD: 21.77 ± 13.09) than in aSLE patients (13.37 ± 8.32) (p = 0.05). Eleven patients died during episodes of acute pancreatitis and mortality rate was significantly higher in pSLE than in the aSLE group (53.8% and 14.8%, respectively, p = 0.015). Mortality was associated with concurrent SLE symptoms (p = 0.049), higher SLEDAI score at presentation of acute pancreatitis (p = 0.011), severe acute pancreatitis (p < 0.001), and the presence of complications (p < 0.001). The multivariate logistic regression analysis showed that severity of acute pancreatitis was the most powerful risk factor for mortality in SLE patients with this disease. In summary, our results indicate that acute pancreatitis occurs more frequently, tends to be more severe, and is associated with higher mortality in pSLE patients when compared with aSLE patient.

Systemic lupus erythematosus (SLE) predominantly affects women of childbearing age, but 15-20% of cases are diagnosed during childhood. It is important for physicians to understand the epidemiology and clinical presentation for early detection and diagnosis of this disease in difference races. The aim of this retrospective review was to provide a 20-year experience for initial clinical and laboratory manifestations and outcomes in pediatric-onset SLE (pSLE) in a medical center in Asia. We reviewed medical records between April 1990 and June 2012 of patients with a diagnosis of International Classification of Diseases, Ninth Revision (ICD-9) code 710.0 (SLE), who admitted or received follow-up in the Department of Pediatrics at Chang Chung Memorial Hospital. Patients with a diagnosis of SLE prior to their 18th birthday and followed up at our hospital were eligible for inclusion in this study. Medical records regarding age, gender, date of birth and diagnosis, clinical manifestations at diagnosis, laboratory results, image studies and the classification criteria were reviewed. Patients received regular outpatient department follow-up and laboratory survey every 1-6 months. The study cohort consisted of 189 patients; 164 females (86.87%) and 25 males (13.23%). The overall mean age at pSLE diagnosis was 12.62 ± 2.77 years. The most common clinical symptom was malar rash, followed by arthritis and oral ulcers. There was no significant difference in clinical and laboratory manifestations between females and males. More than half of the patients presented with renal involvement initially. The most common histological finding was Class IV lupus nephritis (LN), especially in males (p = 0.034) and young age. Even with severe LN, the rate of end-stage renal disease (ESRD) was low if adequate treatment was initiated. The 5, 10 and 15-year ESRD-free survival rates were 95.4%, 94.0% and 89.9% in patients with biopsy-proven LN. However, infection was the leading cause of mortality. Therefore, aggressive treatment for major organ involvement is important, but physicians must also be aware of fatal infection. The overall survival rates were 5 years: 93.4% and 10-20 years: 89.6%.