不典型抗中性粒细胞胞质抗体阳性伴肾脏受累的临床分析

Atypical Anti-neutrophil Cytoplasmic Antibodies Positive Patients with Renal Impairment

目的:研究不典型抗中性粒细胞胞质抗体(A鄄ANCA)阳性伴肾脏受累者的临床和病理特点。方法:以间接免疫荧光(IIF)法作初筛,阳性及可疑血清经酶联免疫吸附试验(ELISA)法分别检测蛋白酶3(PR3)和髓过氧化物酶(MPO)两种靶抗原特异性。结果:肾脏受累伴A鄄ANCA阳性患者6例,男性3例(58～72岁),女性3例(28～40岁),IIF法示细胞内弥漫性细颗粒状荧光亮染,MPO鄄ANCA及PR3鄄ANCA均阴性。多系统受累,肾小管功能不同程度受累,2例肾损均见于男性,4/6例合并肝脏受累。结论:①男性发病年龄晚,临床表现重,部分伴肾功能受累。②多脏器损害以肝脏受累尤为突出,肺部、肾脏受累比例及程度远较MPO鄄ANCA及PR3鄄ANCA阳性患者小而轻。③肾脏损害无特异性,肾小管功能轻度异常较多见。④患者自身免疫功能紊乱,多项免疫指标异常,不排除GS鄄ANA与ANCA部分交叉现象。⑤靶抗原与MPO、PR3关联程度较少,临床、病理表现存在差异。

Objective:To study the clinical feature in patients with atypical anti-ne utrophil cytoplasmic antibodies(A-ANCA)positive and renal impariment.Methods:Po sitive and suspected positive serums determined by an inderect immunofluo-resce nt(IIF)technique,as an initial screening method,were subjected to ELISA tests fo r target antigens of myeloperoxi-dase(MPO)and proteinase3(PR3).Results:Six pati ents with renal impairment were A-ANCA positve,and of them,3were men(58-72year s old)and3were women(28-40years old).IIF technique showed that there was scatte red immunofluores-cent positive particles,while the MPO-ANCA and PR3-ANCA wer e all negative.It was showed that multiple organs were involved and most cases w ere accompanied with different degrees of abnormal renal interstitial function.T wo men present-ed renal insufficiency.Four cases were found liver impairment.Co nclusion:①It may be worse in male patients with lat-er-onset and some with re nal impairment.②Liver lesions are prominent,while lung and kidney were less inv olved.③There is no specificity of renal impairment,except abnormal renal inters tistial function.④Since abnormalities of immune systems are common,cross reacti ng antibodies might exist possibly.⑤MPO and PR3are not confirmed to be associat ed with A-ANCA,as well as differences between the clinical and pathologic findi ngs exist.