儿童系统性红斑狼疮98例临床分析

目的探讨儿童系统性红斑狼疮的临床及实验室特点。方法总结1999年1月至2008年12月在我院内科住院治疗的98例儿童系统性红斑狼疮患者的临床资料。结果98例患儿中,男20例,女78例,男女比例为1：3.90;年龄1～18岁,平均（11.52±3.47）岁;﹤5岁患儿男女比例为1：1,5～10岁男女比例为1：3.75,10～18岁男女比例为1：4.62。首发症状以皮疹最为常见,41例,占41.84%;其次为发热,40例,占40.82%;关节痛13例,占13.27%。系统损害中最常受累的是血液系统,79例,占80.61%;肾脏69例,占70.41%;神经系统占53例,54.08%。血液、肾脏、神经系统受累最常见的表现分别为贫血63例（64.29%）,蛋白尿64例（65.31%）,头晕、头痛10例（10.20%）;实验室检查ANA阳性率最高,占98.98%,其次为抗dsDNA抗体,占64.89%。女性抗rRNP抗体阳性检出率高于男性（掊2=9.00,P﹤0.005）,男性多种病原学阳性率高于女性（掊2=7.45,P﹤0.01）。男女在发病年龄、首发症状、系统损害及其他实验室检查方面均无显著性差异。结论儿童系统性红斑狼疮临床表现多样,为多系统受累,不同性别临床表现及实验室检查存在一定差异。

Clinical analysis of 98 cases of pediatric systemic lupus erythematosus

Objective To investigate the clinical and laboratory characteristics of systemic lupus erythematosus （SLE） in children. Methods The records of children with SLE treated in the Department of Medical of Capital Institute of Pediatrics in the past ten years （between January 1999 to December 2008） were reviewed. Results In 98 children, 78 were girls and 20 were boys and the female- to- male ratio was 3.90：1 overall, with a ratio of 1.00：1 under 5 years, 3.75：1 under 10 years and 4.62：1 over 10 years, respectively. The youngest was 1 year while the oldest was 18 years with the mean age of 11.52±3.47 years. Skin eruption was the most common initial manifestation （41.84%） followed by fever （40.82%） and arthralgia （13.27%）. Systemic involvement was common with 80.61% hematological abnormalities, 70.41% renal involvement and 54.08% nervous system involvement. The most common presentation of hematological, renal and nervous involvement was anemia（64.29%）, proteinuria（65.31%） and dizziness,headache（10.20%）, respectively. Among the laboratory parameters tested anti-nuclear antibody （ANA） was shown to have the highest positive rate of 98.98%, followed by anti-double stranded DNA（dsDNA） with a positive rate of 64.89%. The positive rate of anti-rRNP in female was higher than that in male, while the positive rate of multiple aetiology in male was higher than that in female. There was no significant difference between the male and female patients in the age of onset, initial manifestation, systemic involvement and other laboratory results. Conclusions The manifestations of pediatric SLE are various and multisystem involvement is common. There is gender difference in clinical manifestations and laboratory results.