目的 探讨贝利尤单抗治疗儿童系统性红斑狼疮（SLE）的疗效及安全性。方法 回顾性分析2020年1月1日-2021年12月31日完成贝利尤单抗28周治疗的SLE患儿的临床资料。结果 共纳入17例患儿,男5例、女12例,年龄（12.1±2.3）岁;开始靶向药物治疗的中位病程为5.0（1.0~22.0）月。与治疗前相比,治疗后患儿皮疹、发热、狼疮肾炎和血液系统损伤的比例下降,CD3⁺、CD4⁺细胞计数增加,CD19⁺细胞计数减少,抗ds-DNA阳性率下降,糖皮质激素用量减少,差异有统计学意义（P<0.05）。贝利尤单抗治疗不同时间点（0、2、4、8、12、16、20、24、28周）的系统性红斑狼疮疾病活动指数（SLEDAI-2000）差异有统计学意义（P<0.01）,评分呈现下降趋势。在17例SLE患儿中,5例（29.4%）至观察终点（28周）达到狼疮低疾病活动状态（LLDAS）,3例（17.6%）达到临床缓解。研究期间无严重感染发生,无贝利尤单抗过敏反应。结论 贝利尤单抗联合传统药物治疗SLE可能有助于在不增加不良反应的情况下促使患儿更容易达到LLDAS和临床缓解状态。

Objective To investigate the efficacy and safety of belizumab in the treatment of children with systemic lupus erythematosus (SLE). Methods The clinical data of SLE children who completed 28-week treatment with beliumab from January 1, 2020 to December 31, 2021 were retrospectively analyzed. Results A total of 17 children (5 boys and 12 girls) were enrolled, and the average age was (12.1±2.3) years. The median disease duration from onset to targeted drug therapy was 5.0 (1.0-22.0) months. Compared with the pre-treatment group, the proportion of rash, fever, lupus nephritis and blood system damage decreased, the CD3⁺ and CD4⁺ cell counts increased, the CD19⁺ cell counts decreased, the positive rate of anti-ds-DNA decreased, and the dosage of glucocorticoids decreased in the post-treatment group, and the differences were statistically significant (P<0.05). There was significant difference in the systemic lupus erythematosus disease activity index (SLEDAI-2000) at different time points (weeks 0, 2, 4, 8, 12, 16, 20, 24 and 28) after beliumab treatment (P<0.01), and the score showed a downward trend. Among the 17 SLE children, 5 (29.4%) achieved lupus low disease activity state (LLDAS) and 3 (17.6%) achieved clinical remission by the end of observation (week 28). There was no complication of serious infection and no allergic reaction to belizumab. Conclusions Beliumab combined with traditional drugs in the treatment of SLE may help children more easily achieve LLDAS and clinical remission without severe adverse effects.