40例儿童狼疮性肾炎临床与病理分析

Clinical and immunological features of lupus nephritis in children：retrospective analysis of 40 cases

摘要:

目的 探讨儿童狼疮性肾炎（LN）的临床与病理特点。方法 回顾性分析2005年1月至2010年12月住院治疗的40例LN患儿的临床和病理资料。结果 40例LN患儿中，男4例，女36例，发病年龄2.6~14.3岁，平均10.6±2.6岁，其中学龄儿童35例（88%）。发病至确诊时间范围约3 d~1年左右。40例患儿均存在蛋白尿，其中肾病范围蛋白尿12例（30%），孤立性蛋白尿9例（22%）；伴不同程度血尿患儿26例（65%）。临床分型以急性肾炎型为主（19例，47%）。39例行肾穿刺活检，其中3例未分型，余36例中，Ⅳ型LN 18例（50%），Ⅱ型LN 8例（22%）；抗核抗体在所有病理分型患儿中的阳性率均为100%，抗dsDNA阳性率为61%，89%的患儿血补体C3及C4均有不同程度下降。在疾病治疗6个月时LN缓解率高达95%；Ⅳ、Ⅴ+Ⅲ及Ⅴ+Ⅳ型LN患儿肾脏急性活动指数较其他型明显偏高，慢性指数及肾小管间质损害在不同病理分型患儿间无明显差异。结论 患儿LN的临床表现具有多样性，临床分型以急性肾炎型最多，病理分型以Ⅳ型最为常见，肾脏急性活动指数在Ⅳ、Ⅴ+Ⅲ及Ⅴ+Ⅳ型中偏高，经过6个月的正规治疗，大部分患儿可达到临床缓解，坚持长期规范化治疗有助于改善预后。

Abstract:

Objective To analyze the clinical and immunological features of children with lupus nephritis (LN). Methods Chart records of 40 (4 male and 36 female) LN children who were admitted consecutively between January, 2005 and December, 2010 were reviewed. The baseline demographic, pathological and immunological data were analyzed. Results In the 40 LN patients analyzed, the mean age of the disease onset was 10.6±2.6 (range from 2.6 to 14.3) years, and 35 cases (88%) were school-age children. Proteinuria was detected in all 40 cases, including nephrotic-range proteinuria in 12 (30%) cases, and isolated proteinuria in 9 (22%) cases. Twenty-six (65%) patients had varying degrees of hematuria. Acute nephritis was the most common sub-type, accounting for 47% of the total cases. Among the 39 cases undergoing renal biopsy, 3 were unclassified and the remaining 36 were classified, respectively, as type Ⅳ LN (50%, 18 cases), type Ⅱ LN (22%, 8 cases). In the histopathologcally classified case, 100% were antinuclear antibody-positive, 61% were anti-dsDNA-positive, and 89% showed varying degrees of decrease in serum C3 and C4 concentrations. Following treatment for 6 months, a high LN remission rate (95%) was achieved; the acute renal activity index remained higher in Ⅳ, V+Ⅲ and V+Ⅳ subtypes than in other subtypes, while the chronic index and the degree of tubulointerstitial damage were not different between histopathological subtypes. Conclusions The clinical manifestations of LN children are diverse. Clinically, acute nephritis is the most common form of LN in children. Histopathologically, type Ⅳ is the most frequent subtype of LN. Early treatment may result in significant disease remission.