儿童狼疮性肾炎91例临床和病理分析

Clinical and Pathological Analysis of 91 Children with Lupus Nephritis

目的探讨儿童狼疮性肾炎（lupus nephritis，LN）的临床与病理特点。方法同顾性分析住院治疗的91例儿童LN的临床和病理资料。结果91例LN患儿中男22例，女69例。男女比1：3．1；起病年龄6．0～155岁。临床表现为肾病综合征占44．0％；59例行肾穿刺活检。Ⅳ型LN占59．3％。免疫荧光呈“满堂亮”者占72．0％。6例肾脏免疫荧光不典型，其中3例IgM沉积为主，1例IgA为主。2例呈“寡免疫”。3例临床表现与肾脏病变程度不平行，临床为肾病综合征，病理为Ⅱ型。本组在疾病初期LN缓解率高达937％，随访271％反复，多与小坚持正规治疗有关。结论LN患儿的临床表现和肾脏病理复杂多变，部分患儿很不典型，考虑与其特殊的发病机制有关；早期积极和坚持长规范化治疗对于改善LN的预后非常重要。

Obiective To analyze the clinical and pathological characteristics of children with lupus nephritis（LN）. Methods Ninety - one children with LN were diagnosed from 1993 to 2005, according to the clinical literature and renal pathology and the data were retrospectively summarized. Results Within the cohort of 91 children, there were 69 females and 22 males（female to male ratio 3.1 ）. Most of the sick children were at the school age ranging from 6.0 to 15.5 years old. Nephrotic syndrome（44.0 % ） was the most common clinical manifestation. Fifty - nine renal biopsies were performed. Class Ⅳ- LN（59.3% ） was the most frequent pathological findings. ＂A fullhouse pattern＂ on immunofloorescence was found in 72.0 % of biopsies. The clinical and pathological manifestations of some children were atypical. There were 3 patients characterized by predominant deposits of immunoglobulin M（IgM）, 1 patient with predominant deposits of IgA,and 2 children with pauei- immune LN.Three children with class Ⅱ LN in our study presented with nephrotie syndrome. LN was initially controlled by aggressive treatment in 93.7% of thse patients. Relapses of nephritis covered 27.1% of them, mostly caused by the intermittent treatment. Conclusions The clinical and pathological manifestations of LN were variable. Some atypical LN was considered to be associated with the distinct pathogenesis. Most of LN could be controlled by aggressive treatment. Long and regular treatment is necessary to improve the prognosis of LN.