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1、Glomerular DiseasesFor Internal Medicine1 1医学医学The peripheral portion of a glomerular lobule2 2医学医学Glomerular Diseases ClassificationlPrimarylSecondarylHereditary3 3医学医学Pathogenesis4 4医学医学Immunologic glomerular injuryHumoral antibody-mediatedCellular antibody-independent 5 5医学医学Antibody-mediatedCirc
2、ulating autoantibodies with intrinsic autoantigens: eg. anti-GBM diseaseIn situ formation of immune complexs/ circulationg antibodies with extrinsic antigens that have been “planted” within the glomerulus: eg. Postinfectious glomerulonephritisANCA/AECA associated: no disernible immune complexes in t
3、he glomerular parenchyma6 6医学医学Cellular antibody-independent glomerular injuryLess well definedInitiators of injury in pauci-immune glomerulonephritis, which share the downstream mediators with the antibody- dependent injurySoluble factors from T cells: in MCD and primary FSGS7 7医学医学Nonimmunologic g
4、lomerular injuryMetabolicHemodynamictoxic8 8医学医学immunologic humoral cellularnon-immunologicinflammationGlomerular injury9 9医学医学Clinicopathologic correlates in glomerular disease1010医学医学Major clinicopathologic entities (contd)Nephrotic syndromelGlomerular filtration barrier affectedlNephrotic-range p
5、roteinuria =3.5g/24h lHypoalbuminemia, edema, hyperlipidemia, and lipiduria, and a prothrombotic statelMembranous glomerulopathylMinimal change disease (MCD)lFSGSlMembranoproliferative: hybrid lesion of nephritic and nephrotic features1111医学医学OthersGlomerular deposition diseases: extravascular depos
6、ition of paraprotein or fibrillar materialThrombotic microangiopathies: thrombi within the renal microvasculature1212医学医学Primary insultInflammatoryMetabolic Hemodynamic or mechanic ToxicInfectiousMay overlapMay induce similar clinicopahtologic presentations1313医学医学病变部位系膜 mesangium 系膜细胞mesangial cell
7、系膜基质mesangial matrix基膜 basement membrane上皮细胞 足细胞 podocyte、足突 foot process内皮细胞1414医学医学The peripheral portion of a glomerular lobule1515医学医学基本病变增生 proliferation硬化 sclerosis1616医学医学1.轻微肾小球病变（Minor Lesion)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 minimal change disease，MCD轻度系膜增殖性肾小球肾炎毛细血管内增殖性肾小球肾炎恢复期其它1717医学医学MC
8、D （左）正常，（右）上皮细胞足突广泛融合、消失1818医学医学2. 局灶节段性病变(1)局灶节段性增殖性肾小球肾炎focal and segmental proliferative glomerulonephritis (2)局灶节段性肾小球硬化focal and segmental glomerulosclerosis,FSGS1919医学医学局灶性肾小球肾炎2020医学医学3.弥漫性肾小球肾炎(diffusive glomerulonephritis)(1)膜性肾病 membranous nephropathy, MN肾小球基底膜2121医学医学membranous nephropath
9、y （左）正常，（右）上皮下免役 复合物沉积（D），GBM增厚，钉突形成（S），上皮细胞足突融合2222医学医学(2)增殖性肾小球肾炎 proliferative glomerulonephritis 系膜增殖性肾小球肾炎 mesangial proliferative glomerulonephritisMsPGN肾小球系膜 IgA肾病 IgA nephropathy 非IgA肾病 IgG沉积为主IgM肾病2323医学医学mesangialmesangial proliferative proliferative glomerulonephritisglomerulonephritis （左
10、）正常，（右）系膜细胞和基质增生，电子致密物（D）沉积2424医学医学 毛细血管内增殖性肾小球肾炎 endocapillary proliferative glomerulonephritis 系膜+内皮细胞2525医学医学endocapillary proliferative glomerulonephritis （左） 正常，（右）内皮（E）和系膜（M）细胞增生，上皮下驼峰状 电子致密物（D）沉积2626医学医学 系膜毛细血管性肾小球肾炎 mesangiocapillary glomerulonephritis又称膜增殖性肾小球肾炎 membranoproliferative glomer
11、ulonephritis系膜+基底膜致密沉积物性肾小球肾炎 dense desposit glomerulonephritis电子致密沉积物2727医学医学mesangiocapillarymesangiocapillary glomerulonephritisglomerulonephritis （左）正常，（ 右）系膜增生（M），电子致密物（D），广泛插入（I）2828医学医学 新月体性肾小球肾炎 cresentic glomerulonephritis又称毛细血管外肾小球肾炎 extracapillary glomerulonephritis 肾小球囊上皮细胞(3) 硬化性肾小球肾炎sc
12、lerosing glomerulonephritis2929医学医学cresentic glomerulonephritis （左）正常，（右）GBM断裂， 纤维蛋白漏出(F)，上皮细胞增生(E)，单核巨噬细胞浸润(P)，新 月体形成3030医学医学4.未分类的肾小球肾炎unclassified glomerulonephritis3131医学医学Clinical presentations3232医学医学Clinical classificationAcute glomerulonephritis,AGNRapidly progressive glomerulonephritis, RPG
13、NChonic glomerulonephritis,CGNNephrotic syndrome,NSLatent glomerulonephritis,asymptomatic hematuria and/or proteinuria3333医学医学Acute nephritic syndromeSudden onset (days to weeks)Nephritic urinary sedimentlHematuria:Red blood casts, dysmorphic red blood cellslSubnephrotic proteinuria (20% adults may
14、have persistent proteinuria and/or compromise of GFR3939医学医学RPGNOver weeks to monthsNephritic urinary sediment, subnephrotic proteinuria and variable oliguria, hypervolemia, edema, and hypertensionCrescentic GNCrenscents can also develop concomitantly with proliferative GN, membranous GN and other GN4040医学医学RPGN-Immunofluorescence microscopyanti-GBM dis-more discrete linear deposition of Ig along the GBMimmune complex GN-scattered granular deposits of immunoglobulinpauci-immune GN-paucity or absence of Ig4141医学医学RPGN-Serologic markersDepressed C3 level -Type II
