1Division of Allergy Asthma and Rheumatology, Department of Pediatrics, Chang Gung Memorial Hospital and College of Medicine, Chang Gung University, Taoyuan, Taiwan.
Lupus. 2013 Dec;22(14):1534-40. doi: 10.1177/0961203313502110. Epub 2013 Aug 21.
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%.
系统性红斑狼疮（SLE）主要影响育龄妇女，但 15-20%的病例发生在儿童期。了解不同种族中该病的流行病学和临床表现对于早期发现和诊断非常重要。本回顾性研究的目的是提供亚洲某医学中心 20 年来儿科发病的系统性红斑狼疮（pSLE）的初始临床和实验室表现及结局。我们回顾了 1990 年 4 月至 2012 年 6 月期间在长庚纪念医院儿科就诊或接受随访的国际疾病分类，第 9 版（ICD-9）代码 710.0（SLE）患者的病历。在我们医院确诊为 SLE 且年龄在 18 岁以下的患者符合本研究纳入标准。我们查阅了患者的年龄、性别、出生日期和诊断日期、确诊时的临床表现、实验室结果、影像学研究和分类标准等病历资料。患者在门诊接受定期随访和实验室检查，每 1-6 个月一次。研究队列包括 189 名患者；其中 164 名女性（86.87%），25 名男性（13.23%）。pSLE 诊断时的平均年龄为 12.62 ± 2.77 岁。最常见的临床症状是蝶形红斑，其次是关节炎和口腔溃疡。女性和男性之间的临床表现和实验室检查没有显著差异。超过一半的患者最初表现为肾脏受累。最常见的组织学表现是狼疮肾炎（LN）IV 级，尤其是男性（p = 0.034）和年轻患者。即使出现严重 LN，如果及时开始适当治疗，终末期肾病（ESRD）的发生率也较低。活检证实 LN 的患者 5、10 和 15 年 ESRD 无生存率分别为 95.4%、94.0%和 89.9%。然而，感染是导致死亡的主要原因。因此，积极治疗主要器官受累非常重要，但医生还必须意识到致命感染的风险。总生存率为 5 年：93.4%，10-20 年：89.6%。
