抗磷脂抗体与儿童狼疮性肾炎

Antiphospholipid antibody and childhood lupus nephritis

目的探讨儿童狼疮性肾炎(LN)抗磷脂抗体的阳性率及其与肾脏损害的临床表现/病理分型的关系。方法回顾性分析1997年—2009年确诊LN同时送检抗磷脂抗体的住院患儿的临床和病理资料。结果共50例患儿符合纳入标准,男11例、女39例,平均就诊年龄(11.8±2.1)岁。全部患儿均检测狼疮抗凝物(LA)和抗心磷脂抗体(aCL),31例检测抗β2糖蛋白I(β2-GPI)抗体,其中抗磷脂抗体阳性44例(88.0%),LA、aCL和抗β2-GPI抗体的阳性率分别为80.0%、52.0%和67.7%。抗磷脂抗体阳性与阴性的LN患儿相比,肾病水平蛋白尿、肉眼血尿、高血压和肾功能异常的差异无统计学意义(P均>0.05)。44例患儿进行了肾脏活检,抗磷脂抗体阳性和阴性LN患儿的病理分型差异无统计学意义(P=0.322),Ⅴ型LN仅见于抗磷脂抗体阳性的病例;肾小球微血栓的发生率为20.5%(9/44例),其中18.2%(8/44例)检测抗磷脂抗体阳性。抗磷脂抗体阳性患儿血小板减少的发生率为47.7%,阴性者33.3%(P=0.674)。3例患儿有肾外血栓形成,2例血栓形成在甲基泼尼松龙冲击、中心静脉置管之后,2例在抗磷脂抗体阳性8个月和2年8个月后形成血栓。结论儿童LN中抗磷脂抗体阳性率较高,抗磷脂抗体与LN患儿的临床表现和肾脏病理分型无关,抗磷脂抗体可能是导致肾小球微血栓的主要原因,激素(大剂量)和中心静脉置管是抗磷脂抗体阳性LN患儿形成血栓的可能诱因。

Objective To investigate the prevalence of antiphospholipid antibody（aPL）in childhood lupus nephritis（LN）,and the relationship of aPL with clinicopathological characteristics of LN.Methods The results of aPL tested and pathological data examined were retrospectively analyzed in 50 hospitalized children with LN consisting of 11 boys and 39 girls during Aug.1997 to Dec.2009.Results The mean age of 50 children with LN was 11.8±2.1 years（range：6.0-16.3 yrs）.All children were tested for lupus anticoagulant（LA）and anticardiolipin（aCL）,and 31/50 cases（62%）tested for anti-β2 glycoprotein I（β2GPI）antibody.The positive rates of aPL,LA,aCL and anti-β2GPI antibody were 88.0%,80.0%,52.0% and 67.7%,respectively.No significant differences were found in the prevalence of nephrotic proteinuria,macroscopic hematuria,hypertension and renal failure between aPL-positive and aPL-negative group（P0.05 for all）.Forty-four children underwent renal biopsy.There was no significant difference in the distribution of ISN/RPS classification between the aPL-positive and negative children（P=0.322）.LN class Ⅴ were just found in aPL-positive children.Glomerular microthrombosis were found in 9 out of 44 cases with renal biopsy,mostly aPL-positive（8 cases）.The incidence of thrombocytopenia was higher in aPL-positive children than that in aPL-negative children（47.7% vs.33.3%,P=0.674）.Three children were complicated by thrombosis which occurred in two cases of them after the use of pulse methylprednisolone and central venous catheterization.The duration from the detection of positive aPL to the occurrence of thrombosis were 8 months and 32 months in two children,respectively.Conclusions There is a higher positive rate of aPL in childhood LN.No significant association is found between aPL and clinicopathological characteristics of LN.aPL is probably the major reason to induce glomerular microthrombosis.The pulse methylprednisolone and central venous catheterization can probably be the inducement of thrombosis.