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1、肾脏病与透析肾移植杂志第19卷第3期2010年06月·225·原发性干燥综合征肾损害的临床病理特征及预后刘正钊胡伟新章海涛鲍浩刘志红摘要目的:分析原发性干燥综合征患者肾损害的临床表现、免疫学特点、肾脏病理改变和预后。方法:回顾性分析62例原发性干燥综合征[女60例,男2例,平均年龄(3618±1017)岁]伴肾损害患者的临床表现和肾脏病理特征及预后。结果:8811%患者尿渗量减低,49例(79%)存在肾小管酸中毒(RTA),其中I型44例(8918%),II型2例(411%),III型3例(611%)。47例(7518%)存在低钾血症,9例(1415%)
2、存在肾性尿崩症,1例(116%)存在Fanconi综合征。43例(6914%)有蛋白尿,尿蛋白0142～7151g/d[平均(1117±1116)g/d],其中41例(9513%)尿蛋白<2g/d,均为小管性蛋白尿,2例(417%)尿蛋白>315g/d,4例(615%)有镜下血尿,9例(1415%)存在慢性肾功能不全。45例(7216%)存在高球蛋白血症。血清自身抗体阳性率高低依次为:抗SSA(8610%),ANA(7714%),抗SSB(5216%),RF(3915%),抗心磷脂抗体(2116%),抗RNP抗体(1513%)。肾脏病理56例为间质性肾炎
3、,其中6例伴系膜增生及IgA沉积,2例伴肾小球膜性病变,6例肾小管间质及小球基本正常。56例平均随访(3715±3810)个月(中位随访时间21个月),5年、10年人肾存活率均为100%。结论:原发性干燥综合征肾损害以尿浓缩功能减低和RTA伴低钾血症最为常见,肾脏病理主要为间质性肾炎,少部分患者可有系膜增生伴IgA沉积、肾小球膜性病变,远期预后较好。关键词干燥综合征肾活检临床病理ClinicopathologiccharacteristicsandprognosisinprimarySjÊgrenpssyndromewithrenalinvolvemen
4、tLIUZheng2zhao,HUWei2xin,ZHANGHai2tao,BAOHao,LIUZhi2hongResearchInstituteofNephrology,JinlingHospital,NanjingUniversitySchoolofMedicine,Nanjing210002,ChinaCorrespondingauthor:HUWei2xin(E2mail:huwx64@medmail.com.cn)ABSTRACTObjective:Toanalyzeclinicalmanifestations,immunologicalcha
5、racteristics,pathologicalchangesandprognosisinprimarySjÊgrenpssyndromepatientswithrenalinvolvement.Methodology:Sixty2twoprimarySjÊgrenpssyndromepatientswithrenalinvolment,female/male60/2casesandaverageageof(3618±1017)yearsold,wereenrolledinthisretrospec2tivestudy.Theirclinicalman
6、ifestations,renalpathologicalfeaturesandprognosiswereanalyzed.Results:8811%casesshowedadecreaseinurineosmosis,49cases(79%)renaltubularacidosis(RTA),includingtypeI44cases(8918%),typeII2cases(411%)andtypeIII3cases(611%).47cases(7518%)presentedwithhypokalemia.9cases(1415%)withnephro
7、genicdiabetesinsipidusandonecase(116%)withFanconisyndrome.43cases(6914%)hadurinaryproteinexcretionfrom0142～7151g/d,ofwhich41(9513%)hadproteinurialessthan2g/d,and2(417%)morethan315g/d.4cases(615%)hadmicroscopichematuria,9(1415%)chronicrenalinsufficiency,and45(7216%)hadhyperglobuli
8、naemia.Thepositiverateofserumautoantibod
