儿童首发系统性红斑狼疮与自身免疫性溶血性贫血的临床研究

摘要

目的 研究自身免疫性溶血性贫血(AIHA)在儿童首发系统性红斑狼疮(c-SLE)中的发病率及其临床表现和实验室指标.方法 收集89例c-SLE患儿,其中AIHA 9例,AIHA的诊断需结合临床表现和实验室指标.结果 AIHA占儿童c-SLE的10%(9/89),存在AIHA与不存在AIHA的c-SLE患儿相比较,前者确诊年龄(12岁比14岁,P=0.019)、颧部红斑(11%比59%,P=0.018)、红细胞计数[1.66(1.27~4.7)×1012/L比3.82(1.71~5.38)×1012/L,P=0.007]、血红蛋白[78(51~100)g/L比107(57~146)g/L,P=0.001]、淋巴细胞数[2.0(1.2~2.6)×109/L比1.3(0.2~5.5)×109/L,P=0.019]均明显低于后者.而两者在男女比例、拖延时间、是否为第一胎、多器官受累、自身抗体(ANA、抗ds-DNA、抗-SSA、抗SSB、抗Sm、抗ACL-IgG、抗ACL-IgM、抗Β2-GP1)、补体(C3、C4)、EB-IgG、CMV-IgG、SLEDAI评分、肾炎及其分型方面比较差异均未见统计学意义(P均>0.05).结论 AIHA作为儿童c-SLE的一种少见并且严重的临床表现,由于其缺乏颧部红斑等系统性红斑狼疮典型的表现,容易误诊.因此,所有存在血细胞减少的c-SLE患儿初诊时都应该常规筛查AIHA.%Objective To summarize the characteristics of clinical features, laboratory tests and incidence of autoimmune hemolytic anemia(AIHA) in childhood-onset systemic lupus erythematosus (c-SLE).Methods A retrospective study including 89 children with c-SLE, among them, 9 cases of AIHA.AIHA was diagnosed by combination of the clinical manifestations and laboratory tests.Results AIHA was observed 10% (9/89) in c-SLE children.The differences of female gender, delay time, whether or not the first child, multiorgan involvement, autoantibodies profile, nephritis and its classification, low complement, EB-IgG, CMV-IgG and SLEDAI had no significant between c-SLE children with or without AIHA (P>0.05).c-SLE children with AIHA had a lower frequency of age at diagnosis(12 years old vs.14 years old, P=0.019), malar rash(11% vs.59%,P=0.018), red blood cell[1.66(1.27-4.7)×1012/L vs.3.82(1.71-5.38)×1012/L,P=0.007], hemoglobin[78(51-100)g/L vs.107(57-146)g/L, P=0.001], lymphocyte count[2.0(1.2-2.6)×109/L vs.1.3(0.2-5.5)×109/L, P=0.019] than c-SLE children without AIHA.Conclusions Our study identified AIHA as a rare and severe initial manifestation of c-SLE.Diagnosis is challenging due to the lack of typical symptoms(malar rash) of lupus and the requirement to exclude infection, malignancy and macrophage activation syndrome and so on.Therefore, it's necessary to screening AIHA in all first-visit c-SLE children presenting a hypocytosis.