儿童原发性IgA肾病免疫、病理特点及预后因素分析

目的探讨儿童原发性IgA肾病(IgAN)的免疫、病理特点及其预后影响因素。方法选择1999年8月-2010年8月本院确诊的48例IgAN儿童,分析其临床资料并进行长期随访,根据随访终点时的临床表现分为A、B、C、D 4种结局作为预后评估指标。采用多因素分析评估影响预后的危险因素。结果 1.体液免疫:29.3%患儿血IgA升高,39.0%血IgG降低;细胞免疫:近50%的患儿细胞免疫指标不正常。2.病理结果:采用Lee氏分级,Ⅱ级25例,Ⅲ级18例,Ⅳ级5例,未发现Ⅰ和Ⅴ级。70.8%伴肾间质受损。免疫荧光以IgA+IgG+IgM沉积者占58.3%,单独IgA沉积者仅占6.3%。3.预后:A级29例(60.4%);B级15例(31.3%);C级4例(8.3%);未发现D级。4.影响预后因素:经Logistic回归分析,病理Ⅳ级与预后有关(P<0.05);高血压、大量蛋白尿和肾衰竭对预后影响无统计学意义。结论儿童IgAN存在免疫紊乱,但预后较好;肾脏病理分级是影响儿童IgAN预后的重要因素。

Immunopathological Characteristics and Prognostic Factors for Primary IgA Nephropathy in Children

Objective To explore the immunopathological characteristics and prognostic factors for primary IgA nephropathy(IgAN) in children. Methods Forty-eight IgAN children′s clinicopathological data were collected and analyzed retrospectively from Aug.1999 to Aug.2010.Clinical follow-up observations were performed in the study.According to the manifestations in the endpoint of follow-up study,the result was divided into 4 grades(A,B,C,D).Multiple factors regression analysis was used to estimate risk factors for prognosis. Results 1.Elevated levels of serum IgA was observed in 29.3% IgAN children and serum IgG level was reduced in 39.0% IgAN children.Cellular immunologic dysfunction was observed in almost 50% patients.2.Lee′s grading system was used for pathological study.Twenty-five patients were of Lee′s gradeⅡ,18 patients were of Lee′s grade Ⅲ,5 patients were of Lee′s grade Ⅳ,but grade Ⅰ and Ⅴ were not found.Interstitial damaged lesion was found in 70.8% patients.Fifty-eight point three percent of patients were characterized by mesangial IgA+IgG+IgM deposition in immunofluorescence examination.Solely IgA deposited was in 6.3% of patients.3.The result of prognosis was that 29 cases(60.4%) were grade A,15 cases(31.3%) were grade B,4 cases(8.3%) were grade C,and no case was grade D.4.Influence factors for prognosis was that Lee′s grade Ⅳ was significantly associated with progressive IgAN by Logistic regression analysis(P<0.05).Hypertension,massive proteinuria and renal failure were not related for the prognosis of IgAN. Conclusions Cellular and humoral immunologic dysfunction are found in IgAN children.The prognosis is better in IgAN children.Pathologic classification is the important independent risk factor for the prognosis of IgAN children.