内科学肾小球疾病-丁小强

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1、GlomerularDiseasesForInternalMedicineTheperipheralportionofaglomerularlobuleGlomerularDiseasesClassificationPrimarySecondaryHereditaryPathogenesisImmunologicglomerularinjuryHumoralantibody-mediatedCellularantibody-independentAntibody-mediatedCirculatingautoantibodieswithintrinsicautoantigens:eg.ant

2、i-GBMdiseaseInsituformationofimmunecomplexs/circulationgantibodieswithextrinsicantigensthathavebeen“planted”withintheglomerulus:eg.PostinfectiousglomerulonephritisANCA/AECAassociated:nodisernibleimmunecomplexesintheglomerularparenchymaCellularantibody-independentglomerularinjuryLesswelldefinedIniti

3、atorsofinjuryinpauci-immuneglomerulonephritis,whichsharethedownstreammediatorswiththeantibody-dependentinjurySolublefactorsfromTcells:inMCDandprimaryFSGSNonimmunologicglomerularinjuryMetabolicHemodynamictoxicimmunologichumoralcellularnon-immunologicinflammationGlomerularinjuryClinicopathologiccorre

4、latesinglomerulardiseaseMajorclinicopathologicentities(cont’d)NephroticsyndromeGlomerularfiltrationbarrieraffectedNephrotic-rangeproteinuria>=3.5g/24hHypoalbuminemia,edema,hyperlipidemia,andlipiduria,andaprothromboticstateMembranousglomerulopathyMinimalchangedisease(MCD)FSGSMembranoproliferative:hy

5、bridlesionofnephriticandnephroticfeaturesOthersGlomerulardepositiondiseases:extravasculardepositionofparaproteinorfibrillarmaterialThromboticmicroangiopathies:thrombiwithintherenalmicrovasculaturePrimaryinsultInflammatory√Metabolic√Hemodynamicormechanic√ToxicInfectiousMayoverlapMayinducesimilarclin

6、icopahtologicpresentations病变部位系膜mesangium系膜细胞mesangialcell系膜基质mesangialmatrix基膜basementmembrane上皮细胞足细胞podocyte、足突footprocess内皮细胞Theperipheralportionofaglomerularlobule基本病变增生proliferation硬化sclerosis1.轻微肾小球病变（MinorLesion)无特异性病变光镜下可见轻度系膜细胞增生和系膜基质增多轻微病变肾病minimalchangedisease，MCD轻度系膜增殖性肾小球肾炎毛细血管内增殖性肾小球

7、肾炎恢复期其它MCD（左）正常，（右）上皮细胞足突广泛融合、消失2.局灶节段性病变(1)局灶节段性增殖性肾小球肾炎focalandsegmentalproliferativeglomerulonephritis(2)局灶节段性肾小球硬化focalandsegmentalglomerulosclerosis,FSGS局灶性肾小球肾炎3.弥漫性肾小球肾炎(diffusiveglomerulonephritis)(