【作者】 张建；

【导师】 封其华；

【作者基本信息】 苏州大学 ， 儿科学， 2019， 硕士

【摘要】 目的分析40例儿童重症系统性红斑狼疮(Systemic lupus erythematosus,SLE)的临床特点,为儿童重症SLE早期诊断提供依据,同时探讨抗中性粒细胞胞浆抗体(ANCA)在重症SLE患儿中检测的临床意义。方法 回顾性分析2009年01月至2018年12月在我院风湿免疫科确诊及首次住院治疗的40例重症系统性红斑狼疮儿童的年龄、临床表现、实验室检查及系统性红斑狼疮疾病活动指数评分资料,分析:1、40例重症SLE患儿根据性别分为男、女两组,比较两组临床表现、实验室检查、SLEDAI评分有无差异。2、收集同期住院治疗的68例轻型SLE患儿临床资料为对照组,分为轻型、重症SLE两组,比较两组实验室检查有无差异。3、根据40例重症SLE患儿检测ANCA值,分为ANCA阳性、ANCA阴性两组,比较两组患儿的实验室检查、肾脏病理分型有无差异。结果 1、40例重症SLE患儿中,男11例,女29例,男女比例为1:2.64,平均发病年龄为10.79±2.74岁,平均发病年龄在男女之间的差异无统计学意义(t=0.465,P>0.05)。2、40例重症SLE患儿重要器官严重受累中严重肾脏损害15例(占37.5%),其中肾病综合征12例、急性肾功能衰竭3例;严重血液系统损害7例(占17.5%),其中溶血性贫血3例、严重粒细胞减少(WBC<1×109/L)1例、严重血小板减少(<50×109/L)3例;严重呼吸系统损害7例(占17.5%),其中肺炎6例、肺出血1例;严重神经系统损害5例(占12.5%),其中昏迷2例、惊厥3例;严重心血管系统损害5例(占12.5%),其中心肌炎3例、心包炎1例、恶性高血压1例;严重消化系统损害1例(占2.5%),为急性胰腺炎。3、40例重症SLE患儿临床表现中出现皮疹27例(占67.5%),发热25例(占62.5%),血液系统损害31例(占77.5%),肾脏损害26例(占65%),淋巴结肿大19例(占47.5%),肝或脾脏肿大13例(占32.5%)。4、血清学检查:自身抗体中抗ANA抗体阳性40例(占100%),抗ds-DNA抗体阳性29例(占72.5%),抗核小体抗体阳性28例(占70.0%),抗SSA抗体阳性19例(占47.5%),抗AHA抗体阳性17例(占42.5%),抗Sm抗体阳性15例(占37.5%),抗rRNP抗体阳性14例(占35.0%),抗SSB抗体阳性7例(占17.5%);免疫球蛋白IgA升高、IgG升高、IgM升高比例依次为87.5%、85.0%、42.5%;补体C4降低35例(占87.5%)、补体C3降低32例(占80.0%)。5、40例重症SLE患儿中有36例完善肾脏穿刺活检,结果显示均存在肾脏损害,其中以狼疮性肾炎Ⅳ型22例(占61.1%)最多见,其次为狼疮性肾炎Ⅱ型、狼疮性肾炎Ⅲ型各6例(均占16.7%),狼疮性肾炎V型为2例(占5.6%),未见狼疮性肾炎Ⅰ型和狼疮性肾炎Ⅵ型。6、重症SLE男性患儿中白细胞减少、中性粒细胞减少、ALT升高、AST升高人数明显多于女性患儿;重症SLE男性患儿SLEDAI评分明显高于女性患儿,两组间比较均有显著性差异(P<0.05)。7、重症SLE组HB、PLT、C3、C4均显著低于轻型SLE组;重症SLE组WBC、LY、ALT、AST、24h尿蛋白、CRP、血肌酐、BUN、铁蛋白均显著高于轻型SLE组;重症SLE组中无菌性脓尿、蛋白尿、血尿、抗dsDNA抗体阳性、抗核小体抗体阳性、ANCA阳性比例均显著高于轻型SLE组,差异均具有统计学意义(均为P<0.05)。8、重症SLEANCA(+)组血清C3、C4、白蛋白水平显著低于重症SLEANCA(-)组;重症SLEANCA(+)组抗dsDNA抗体、抗核小体抗体、无菌性脓尿、血尿、蛋白尿阳性率显著高于重症SLEANCA(-)组;重症SLEANCA(+)组24h尿蛋白、血肌酐、尿素氮、SLEDAI评分显著高于重症SLEANCA(-)组,差异均有统计学意义,P值均小于0.05。9、重症SLEANCA(+)组和重症SLEANCA(-)组在肾脏病理分型构成上有显著性差异,P=0.005,重症SLEANCA(+)组的病理分型较重。结论1、外周血血红蛋白、血小板明显下降,补体C3、C4明显下降,ALT、AST、血肌酐、尿素氮、铁蛋白明显升高,ANCA阳性可提示为儿童重症系统性红斑狼疮。2、对于男性重症SLE患儿,更易合并白细胞特别是中性粒细胞减少、肝功能异常。3、重症SLE患儿ANCA阳性率较高,提示ANCA阳性与重症SLE可能存在一定的相关性,且可提示SLE的活动性。更多还原

【Abstract】 ObjectiveThe clinical features of 40 children with severe systemic lupus erythematosus(SLE)were analyzed to provide the basis for the early diagnosis of severe SLE in children,To investigate the clinical significance of anti-neutrophil cytoplasmic antibody(ANCA)in children with severe SLE.MethodsRetrospective analysis of age,clinical manifestations,laboratory tests and systemic lupus erythematosus activity index scores of 40 children with severe systemic lupus erythematosus diagnosed and first hospitalized in our hospital from January 2009 to December 2018.Analysis:1.According to the gender,divided into severe SLE male and female groups.Comparing the clinical manifestations,laboratory tests,and systemic lupus erythematosus disease activity index scores.2.The clinical data of 68 children with light SLE who were hospitalized during the same period were collected as the control group.They were divided into two groups:light and severe SLE.The differences between the two groups were compared.3.According to the children’s detection of ANCA,they were divided into severe SLE ANCA-positive and severe SLE ANCA-negative groups.The clinical data of the two groups were compared.Result1.Of the 40 children with severe SLE,11 were male and 29 were female.The male to female ratio was 1:2.64,and the mean age of onset was 10.79±2.74 years.There was no significant difference in the average age of onset between men and women(t=0.465,P>0.05.2.Among the 40 cases of severe organ involvement in SLE,15 cases(37.5%)had severe renal damage,including 12 cases of nephrotic syndrome and 3 cases of acute renal failure;Severe blood system damage in 7 cases(17.5%),3 cases of hemolytic anemia,1 case of severe neutropenia(WBC<1×109/L),3 cases of severe thrombocytopenia(<50×109/L);Severe respiratory system damage was found in 7 cases(17.5%),including 6 cases of pneumonia and 1 case of pulmonary hemorrhage;Severe neurological damage was found in 5 cases(12.5%),including 2 cases of coma,3 cases of convulsion.There were 5 cases of severe cardiovascular system(12.5%),including 3 cases of myocarditis,1 case of severe pericarditis,1 case of malignant hypertension.One case(2.5%)of digestive system damage was acute pancreatitis.3.Among the 40 patients with severe SLE,27(67.5%)presented skin rash,25(62.5%)presented fever,31(77.5%)presented blood system damage,26(65%)presented kidney damage,19(47.5%)presented lymph node enlargement,and 13(32.5%)presented liver or spleen enlargement.4.Serological examination:autoantibody in resistance to ANA antibody positive 40 cases(100%),anti ds-DNA antibody positive 29 cases(72.5%),anti nucleosome antibody positive 28 cases(70.0%),anti SSA antibody positive 19 cases(47.5%),anti AHA antibody positive 17 cases(42.5%),anti Sm antibody positive 15 cases(37.5%),anti rRNP antibody positive 14 cases(35.0%),anti SSB antibody positive in 7 cases(17.5%);The increased proportion of IgA,IgG and IgM was 87.5%,85.0%and 42.5%respectively.C4 decreased in 35 cases(87.5%)and C3 decreased in 32 cases(80.0%).5.Thirty-six of the 40 patients with severe SLE a complete renal biopsy,the results showed there were kidney damage,Among them,22 cases(61.1%)were most common in lupus nephritis type IV,followed by lupus nephritis type II and lupus nephritis type Ⅲ in 6 cases(both 16.7%),and 2 cases(5.6%)of lupus nephritis.No lupus nephritis type I and lupus nephritis type VI were found.6.The number of male children with leukopenia and neutropenia and increased ALT and AST was significantly higher than that of female children.The SLEDAI score of male children was significantly higher than that of female children,and there were significant differences between the two groups(P<0.05).7.HB,PLT,C3 and C4 in children with severe SLE were significantly lower than those in children with mild SLE.WBC,LY,ALT,AST,24h urinary protein,CRP,serum creatinine,BUN,and ferritin were significantly higher in children with severe SLE than in children with mild SLE;The incidence of aseptic pyuria,proteinuria,hematuria,anti-dsDNA antibody positive,anti-nucleosome antibody positive,and ANCA positive in children with severe SLE were significantly higher than those in children with mild SLE.The difference was statistically significant(P<0.05).8.The levels of serum C3,C4 and albumin in ANCA(+)group were significantly lower than those in ANCA(-)group;the positive rate of anti-dsDNA antibody,anti-nucleosome antibody,aseptic pyuria,hematuria and proteinuria in ANCA(+)group was significantly higher than ANCA The(-)group;ANCA(+)group 24h urine protein,serum creatinine,urea nitrogen,SLEDAI score was significantly higher than the ANCA(-)group.The differences were statistically significant(P<0.05).9.The severe SLE ANCA(+)group and the severe SLE ANCA(-)group have significant differences in pathological typing composition,P=0.005,and the severe SLE ANCA(+)group has heavier pathological typing.Conclusion:1.Peripheral hemoglobin,blood platelets decreased significantly,complement C3 and C4 decreased significantly,ALT,AST,serum creatinine,urea nitrogen,ferritin increased significantly,ANCA positive may indicate severe systemic lupus erythematosus in children.2.For children with severe SLE in men,it is easier to combine with white blood cells,especially neutropenia and abnormal liver function.3.The positive rate of ANCA in children with severe SLE is high,suggesting that there may be a certain correlation between ANCA positive and severe SLE,and may indicate the activity of SLE.更多还原