儿童系统性小血管炎患者的临床病理特点及预后分析  

Systemic vasculitis in children: clinic-pathological characteristics and outcome

作　　者：李小伟[1,2] 梁少姗[2] 郑春霞[2] 曾彩虹[2] 章海涛[2] 胡伟新[2] 刘志红[1,2] 

机构地区：[1]东南大学临床医学院,南京210009 [2]南京军区南京总医院国家肾脏疾病临床医学研究中心全军肾脏病研究所

出　　处：《肾脏病与透析肾移植杂志》2014年第3期221-228,246,共9页Chinese Journal of Nephrology,Dialysis & Transplantation

基　　金：十二五国家科技支撑计划课题(2011BAI10B04)

摘　　要：目的:通过与成人系统性小血管炎患者的比较,分析儿童系统性小血管炎患者的临床病理特点及血管炎病理分型在儿童患者中的应用价值。方法:选取经肾活检明确诊断为系统性小血管炎的儿童患者38例,通过与同期入院的285例成人患者比较,分析其临床病理特点并对血管炎病理分型在儿童患者中的应用价值进行评估。结果:(1)儿童患者占同期诊断的系统性小血管炎患者11.8%(38/323),与成人患者比较,其女性患者比例高(73.7%vs 51.9%,P<0.05),以显微镜下多血管炎(71.1%)及P-ANCA/抗MPO抗体阳性(63.2%)为主,其分布与成人患者无差异。(2)儿童患者首发症状中皮肤紫癜发生率高(31.6%vs 6.0%,P<0.01)。肾外表现中肺部受累比例低(26.3%vs 46.0%,P<0.05),皮肤受累比例高(36.8%vs 13.7%,P<0.01),两组患者在肾脏受累无差异。(3)实验室检查方面,儿童患者CD8+T淋巴细胞水平较成人患者高(P<0.05),血IgG水平较成人患者低(P<0.05),其他指标均无明显差异。(4)肾脏病理方面,两组在新月体比例、球性硬化比例及肾小管间质损伤中无显著差异(P均>0.05)。儿童患者肾间质PCNA水平较成人高(54.36±108.92 vs 14.98±28.95,P<0.05)。(5)病理分型评估,两组病理分型的构成比无明显差别,新的病理分型在两组中对肾存活率判断均有价值(P均<0.01)。儿童组与成人组在肾存活率上无差异(P>0.05)。结论:儿童患者占同期入院系统性小血管炎患者的11.8%,女性发病率较男性高,首发症状中以皮肤紫癜多见,临床表现中肺部受累比例低与成人。新的病理分型能够较好的对儿童系统性小血管炎患者的预后进行判断。Objective：To investigate the clinic-pathological character（）tics and the predictive value of the histopathologic classification in children with systemic small blood vessel vascalitis （SSV）.Methodology：Thirty pediatric patients diagnosed as SSV were collected,and two hundred eighty five adult SSV cases were regarded as controls.The clinic-pathological features and the predictive value of the histopathologic classification were compared between pediatric and adult groups.All patients had renal biopsy and followed up for more than one year.Results：The pediatric cases accounted for 11.8% （38/323） of SSV patients in the same period.Compared with adult group,the female was predominant in pediatric patients （73.7% vs 51.9%,P〈 0.05）.The main causes of SVV from both groups were microscopic polyangiitis （MPA） （71.1% vs 72.3 %,P〉0.05） and the majority of the sera pattems was P-ANCA/anti-MPO antibody positive （63.2% vs 75.4%,P〉0.05）.For the first symptom,the prevalence of skin purpura in pediatric patients was significantly higher than that in adults （31.6% vs 6.0%,P〈0.01）.For the clinical manifestations,the prevalence of of skin purpura was higher and pulmonary lesion was lower in pediatric than that in adults （36.8% vs 13.7%,26.3% vs 46.0%,P〈0.01,P〈0.05）.There was no significant difference in renal manifestations and laboratory tests such as serum albumin,globulin and baseline creatinine levels except for higher peripheral blood CD8 and lower serum IgG in pediatric than that in adults（all P〈0.05）.There were no significant differences for glomerular,interstitial,or tubular lesions between the two groups except for severe PCNA in renal interstitial in pediatric patients （54.36± 108.92 vs 14.98±28.95,P〈0.05）.The patients of focal subtype presented with significantly lower serum creatinine than that sclerotic subtype （P〈0.01）,crescentic subtype were more likely to respond to treatment and sclerotic subtype patients had worse renal survival in both of pe

关 键 词：儿童 血管炎 临床 病理分型 预后 

分 类 号：R725.9[医药卫生—儿科]