Moradinejad M H, Zamani G R, Kiani A R, Esfahani T
Dep. of Rheumatology, Medical Sciences, University of Tehran, Tehran, Iran.
Acta Reumatol Port. 2008 Jan-Mar;33(1):63-7.
Analysis of the clinical and laboratory features of childhood onset systemic lupus erythematosus (JSLE).
Forty five children, aged below 16, were enrolled in this retrospective multicenter study. All patients fulfilled the American College of Rheumatology revised criteria 1982 for the diagnosis of SLE and had shown clinical manifestations of the disease before the age of 16.
The female to male ratio was 8:1. The mean age at onset was 10.5 (ranged between 3 and 16 years). Thirty patients (66%) were correctly diagnosed before referring to our Center. The clinical manifestation in different organs were as follows: 40 patients (88.8%) had skin involvement, 35 patients (77.7%) experienced musculoskeletal involvement, 29 children (64.4%) suffered from renal disease, hematological abnormalities were detected in 25 patients (55.5%), 12 patients (26%) had cardiovascular disease, 10 patients (17%) presented central nervous system involvement, and 5 patients (11%) experienced SLE-related pulmonary disease. During the follow up period, four patients died, two from renal failure, one from CNS complications of JSLE, and one due to severe sepsis.
Clinical manifestations of Juvenile SLE are diverse and often severe. Similar studies should be undertaken in different geographic areas in order to provide a good insight of the disease towards a correct diagnosis of JSLE.
分析儿童期起病的系统性红斑狼疮（JSLE）的临床和实验室特征。
本回顾性多中心研究纳入了45名16岁以下儿童。所有患者均符合美国风湿病学会1982年修订的SLE诊断标准，且在16岁之前已出现该疾病的临床表现。
男女比例为8:1。发病的平均年龄为10.5岁（范围在3至16岁之间）。30名患者（66%）在转诊至我们中心之前得到了正确诊断。不同器官的临床表现如下：40名患者（88.8%）有皮肤受累，35名患者（77.7%）有肌肉骨骼受累，29名儿童（64.4%）患有肾脏疾病，25名患者（55.5%）检测到血液学异常，12名患者（26%）有心血管疾病，10名患者（17%）出现中枢神经系统受累，5名患者（11%）有与SLE相关的肺部疾病。在随访期间，4名患者死亡，2名死于肾衰竭，1名死于JSLE的中枢神经系统并发症，1名死于严重脓毒症。
青少年SLE的临床表现多样且往往较为严重。应在不同地理区域开展类似研究，以便更好地了解该疾病，从而对JSLE做出正确诊断。
