目的 对不同性别系统性红斑狼疮（SLE）患儿的临床特征及随访情况进行比较。方法 回顾性分析2008~2018年确诊为SLE的≤14岁患儿79例（男18例，女61例）的临床资料，对男女儿童首发及主要临床症状、器官/系统损害情况、相关实验室指标、随访情况等进行对比研究。结果 首发症状及非首发症状中，男童组以发热发生率最高，女童组以面部红斑发生率最高。男童更易出现肾脏和血液系统的损害（P < 0.05），其中蛋白尿发生率较女童明显升高（P < 0.05）。女童更易出现关节痛（P < 0.05）。两组抗核抗体、抗双链DNA抗体、补体C3、红细胞沉降率的异常率均很高（>80%，P > 0.05）。男童组的疾病活动度在首次就诊及随访至第9年时高于女童组（P < 0.05）。随访结果显示（随访时间1个月至10年），在男童组中，3例失访，1例死亡，7例病情控制良好，但均需口服较大剂量激素或免疫抑制控制病情，2例发展为慢性肾功能衰竭，1例出现狼疮脑病。女童组中，3例失访，5例死亡，34例病情控制良好，其中口服醋酸泼尼松10 mg以下维持的有5例，停药1年的1例，停药2年的2例；4例出现狼疮脑病；1例在患病后第7年出现抑郁焦虑状态并有自杀倾向；2例出现视力下降、视物模糊、绿视；1例在服用激素第3年出现双侧股骨头缺血坏死。结论 不同性别SLE患儿的临床特征、部分实验室指标及预后存在差异。男性SLE患儿可能起病更重，更易发生肾脏及血液系统损害，且远期预后可能更差。女性SLE患儿可能更易累及关节。
Abstract
Objective To compare the clinical features and follow-up results of systemic lupus erythematosus (SLE) between boys and girls. Methods A retrospective analysis was performed for the clinical data of 79 children (18 boys and 61 girls), aged ≤ 14 years, who were diagnosed with SLE from 2008 to 2018. The boys and the girls were compared in terms of initial and major clinical symptoms, injury of organs/systems, related laboratory markers, and follow-up results. Results As for the initial and non-initial symptoms, fever had the highest incidence rate in the boys, while facial erythema had the highest incidence rate in the girls. The boys tended to develop renal injury and hematological damage (P < 0.05), with a significantly higher incidence rate of proteinuria than the girls (P < 0.05), while the girls tended to develop joint pain (P < 0.05). There were high abnormal rates (>80%) of anti-nuclear antibody, dsDNA, complement C3, and erythrocyte sedimentation rate in both boys and girls (P > 0.05). The boys had a significantly higher disease activity than the girls at the first visit and in year 9 of follow-up (P < 0.05). A one-month to ten-year follow-up showed that among the boys, 3 were lost to follow-up, 1 died, 7 were well controlled but required oral administration of large doses of hormones or immunosuppression, 2 progressed to chronic renal failure, and 1 developed lupus encephalopathy. Among the girls, 3 were lost to follow-up; 5 died; 34 were well controlled, among whom 5 were maintained on oral prednisone acetate with a dose of <10 mg, 1 was withdrawn from the drug for 1 year, and 2 were withdrawn from the drug for 2 years; 4 developed lupus encephalopathy; 1 developed depression and anxiety and had suicidal tendency in the 7th year after disease onset; 2 experienced impaired vision, blurred vision, and chloropsia; 1 developed a vascular necrosis of both femoral heads in the 3rd year of hormone administration. Conclusions There are differences in clinical features, several laboratory markers, and prognosis between boys and girls with SLE. Boys tend to have a high severity at disease onset, develop renal injury and hematological damage, and have poor long-term prognosis, while girls tend to have joint involvement.
关键词
系统性红斑狼疮 /
性别 /
临床特征 /
随访 /
儿童
Key words
Systemic lupus erythematosus /
Sex /
Clinical feature /
Follow-up /
Child
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基金
2017年宁夏自治区卫计委重点科研项目（2017-NW-003）；宁夏自然科学基金（2019AAC03226）。
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