摘要
目的:分析儿童大疱性系统性红斑狼疮的临床特点。方法:回顾性分析3例儿童大疱性系统性红斑狼疮资料,并总结文献报道的15例病例临床特点及治疗转归。结果:18例患儿中皮肤和肾脏病理资料完整者16例,平均年龄11.22岁,男女比例为2∶16;10例以皮肤大疱首发,12例大疱发生在狼疮肾炎之前;12例合并发热、9例合并口腔溃疡,14例出现贫血。所有病例ANA阳性,13例存在3种及以上血清自身抗体。皮肤病理特点为基底膜补体及免疫球蛋白沉积,10例24 h尿蛋白≥1.5 g;16例患儿具有肾脏病理资料,其中Ⅴ型狼疮肾炎7例、Ⅳ型狼疮肾炎6例、Ⅲ型狼疮肾炎3例。氨苯砜对皮损有效,免疫抑制剂可减少大疱复发,利妥昔单抗和贝利尤单抗治疗对于难治性患儿可能有效。结论:多系统性受累、多种自身抗体阳性和狼疮肾炎是儿童大疱性系统性红斑狼疮的特点,免疫抑制治疗可改善预后,抗B细胞治疗可作为难治性病例的选择。
Objective:To investigate the clinical,diagnostic and therapeutic characteristics of pediatric bullous systemic lupus erythematosus(pediatric BSLE).Methods:The data of three cases with pediatric BSLE in our hospital from 1998 to 2021 and 15 cases reported in literature were analyzed.Results:Of 18 cases,the average age was 11.22 years old,and the male to female ratio was 16∶2.The bullous was the initial symptom in 10 cases and the bullous preceded lupus nephritis in 12 cases.12 cases had fever,9 had oral ulcer and 14 had anemia.The ANA was positive in all patients and 13 patients had simultaneously three or more types of auto-antibodies.The pathological features of skin in 16 cases were the deposition of complement and immunoglobulin in the basement membrane.10 cases had massive urine protein(≥1.5 g/24 hour).Renal pathological data were obtained in 16 cases,7 were classⅤlupus nephritis,6 were classⅣlupus nephritis and 3 were classⅢlupus nephritis.Glucocorticoid,dapsone and immunosuppressive agent were commonly used therapy.Immunosuppressive agent can reduce the recurrence of disease.Rituximab and Belimumab can be used for refractory patients.Conclusion:Children with bullous systemic lupus erythematosus are characterized by multiple systemic involvement,multiple positive autoantibodies and lupus nephritis.Active immunosuppressive therapy can improve the prognosis,and anti-B cell therapy can be used as an option of therapy.
作者
朱丽媛
杨文浩
ZHU Liyun;YANG Wenhao(Department of Rheumatology,Kailuan General Hospital,Tangshan 063000,China)
出处
《中国麻风皮肤病杂志》
2023年第3期151-155,共5页
China Journal of Leprosy and Skin Diseases
关键词
大疱性狼疮
系统性红斑狼疮
临床特点
儿童
bullous lupus
systemic lupus erythematosus
clinical features
pediatric
