《狼疮性肾炎》ppt课件

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1、狼疮性肾炎北京协和医院目录狼疮性肾炎概述增殖性狼疮性肾炎的诱导缓解狼疮性肾炎的维持缓解治疗狼疮性肾炎ESRD的治疗小结系统性红斑狼疮概述SLE的人群发生率1.8-7.6／10万人，患病率为40-200／10万女性与男性之比为10:1男性与女性狼疮，肾炎发生比例相同发病15-45岁，85%以上的患者发病年龄在55岁以前狼疮性肾炎概述在未选择的病例大约有25%-50%在SLE发病时就有临床肾脏疾病，病程中成人中的60%，儿童中的80%的SLE患者会患狼疮性肾炎。LN累计患病率:亚裔为55%、非裔51%、西班牙裔43%、

2、高加索白人14%。非裔、西班亚裔和亚裔往往有更为严重的病例儿童易合并严重的肾炎，而老年人则较少。StichwehD,.CurrOpinRheumatol200416:577–587BogdanovicRPediatrNephrol200419:36–44OrtegaLM,etal:Lupus(2010)19,557–574InternationalSocietyofNephrology/RenalPathologySociety(2003)ClassificationofLupusNephritis(LN)IMin

3、imalmesangialLNIIMesangialproliferativeLNIIIFocalLN*(<50%ofglomeruli)AA/CCIVDiffuseLN*(50%ofglomeruli)Diffusesegmental(IV-S)orglobal(IV-G)LNAA/CCVMembranousLN,III+VIV+VVIAdvancedsclerosingLN(90%globallysclerosedglomeruliwithoutresidualactivity)Lupusnephritiscl

4、assIILupusnephritisclassII.Thereismildglobalmesangialhypercellularity(periodicacid–Schiff,×400).LupusnephritisclassII.Electronmicrographshowingabundantmesangialelectron-densedeposits(×12,000).LupusnephritisclassIIILupusnephritisclassIII.Thereisfocalsegmentalen

5、docapillaryproliferation(Jonesmethenaminesilver,×100).Theglomerularendocapillaryproliferationisdiscretelysegmentalwithnecrotizingfeaturesandanearlycel-lularcrescent(Jonesmethenaminesilver,×400).LupusnephritisclassIVLupusnephritisclassIV.Electronmicrographshow-

6、ingalargesubendothelialelectron-densedepositaswellasafewsmallsubepithelialdeposits(arrow)(×1200).LupusnephritisclassIV.Thereisglobalendocapillarypro-liferationwithinfiltratingneutrophilsandsegmentalwireloopdeposits(hematoxylinandeosin,×320).Lupusnephritisclass

7、VLupusnephritisclassV.Electronmicro-graphshowingnumeroussubepithelialelectron-densedepositsaswellasmesangialdeposits(×5000).LupusnephritisclassV.Silverstainhighlightsglomerularbasementmembranespikesprojectingoutwardfromtheglomerularbase-mentmembranestowardtheu

8、rinaryspace(Jonesmethenaminesilver,×800).其他病理学改变间质－小管性改变血管炎：非炎症性坏死性血管炎，类似于多血管炎，TMA足细胞病：微小病变、FSGS或塌陷性肾小球病的狼疮性肾炎的临床表现活动性沉渣蛋白尿大量蛋白尿NS高血压CrGFRDNA抗体补体其他classI无无或轻微无无classII无<1g/d极少见少见正