1、 徐徐 缓缓 Department of Pathology Department of Pathology West China hospital West China hospital 2014.10 2014.10NephrologyNephrologyUrologyUrologyCT scanCT scan1 12 2Gross viewGross viewNormal nephronNormal nephronFiltering Filtering membranemembrane Size-dependent barrier functionSize-dependent barri
2、er function Charge-dependent barrier Charge-dependent barrier functionfunctionEtiology and PathogenesisEtiology and PathogenesisCirculating Immune Complex Circulating Immune Complex NephritisNephritisnCaused by the trapping of circulating Caused by the trapping of circulating antigen-antibody comple
3、xes within glomeruliantigen-antibody complexes within glomerulinThe antibodies have no immunologic The antibodies have no immunologic specificity for glomerular constituentsspecificity for glomerular constituentsnType hypersensitivity reactionType hypersensitivity reactionnThe antigens may be of end
4、ogenous or The antigens may be of endogenous or exogenous originsexogenous originsCirculating Immune Complex Circulating Immune Complex NephritisNephritisnThe electron-dense deposits lie in the The electron-dense deposits lie in the mesangium,between the endothelial cells mesangium,between the endot
5、helial cells and the GBM(subendothelial deposits)or and the GBM(subendothelial deposits)or between the GBM and the podocytes between the GBM and the podocytes(subepithelial deposits)(subepithelial deposits)nThe deposits may be degraded by monocytes The deposits may be degraded by monocytes and mesan
6、gial cellsand mesangial cellsIn Situ Immune Complex DepositionIn Situ Immune Complex DepositionnAntibodies react directly with intrinsic Antibodies react directly with intrinsic tissue antigen,or antigens“planted”in tissue antigen,or antigens“planted”in the glomeruli from the circulation.the glomeru
7、li from the circulation.nAutoantibodies against components of the Autoantibodies against components of the GBM are the cause of anti-GBMmediated GBM are the cause of anti-GBMmediated disease,often associated with severe disease,often associated with severe injury.The pattern of deposition is injury.
8、The pattern of deposition is linear.linear.nAntibodies may also be formed against Antibodies may also be formed against antigens that are planted in the GBM.The antigens that are planted in the GBM.The resultant in situ immune complexes may resultant in situ immune complexes may show a granular patt
9、ern of deposition.show a granular pattern of deposition.Granular patternGranular patternImmunofluorescence(IF)Immunofluorescence(IF)Linear patternLinear patternImmunofluorescenceImmunofluorescenceCell-Mediated Immunity in GNCell-Mediated Immunity in GNnSensitized nephritogenic T cells,as Sensitized
10、nephritogenic T cells,as a reflection of cell-mediated immune a reflection of cell-mediated immune reaction,cause some forms of reaction,cause some forms of glomerular injury and are involved in glomerular injury and are involved in the progression of many the progression of many glomerulonephrities
11、.glomerulonephrities.nAntibody-mediated immune injury is an Antibody-mediated immune injury is an important mechanism of glomerular damage,important mechanism of glomerular damage,mainly via complement-and leukocyte-mainly via complement-and leukocyte-mediated pathways.Antibodies may also be mediate
12、d pathways.Antibodies may also be directly cytotoxic to cells in the directly cytotoxic to cells in the glomerulus.glomerulus.nThe most common forms of antibody-mediated The most common forms of antibody-mediated GN are caused by the deposition of GN are caused by the deposition of circulating immun
13、e complexescirculating immune complexesDistribution of basic lesionsDistribution of basic lesionsMorphologyMorphologyGross Gross examinationexaminationSwollen with Swollen with scattered petechiascattered petechiaNormal glomerulusNormal glomerulusPost-streptococcal Post-streptococcal glomerulonephri
14、tis(HE)glomerulonephritis(HE)Post-streptococcal Post-streptococcal glomerulonephritis(PAS)glomerulonephritis(PAS)Post-streptococcal glomerulonephritis(PASM)Post-streptococcal glomerulonephritis(PASM)ImmunofluorescenceImmunofluorescenceGranular deposits Granular deposits C3C3SubepitheliaSubepithelial
15、“humps”l“humps”immune immune complexes complexes depositiondepositionElectron Electron microscopymicroscopySubepitheliaSubepithelial“humps”l“humps”immune immune complexes complexes depositiondepositionAcute onsetAcute onset OliguriOliguria a EdemaEdemaHypertensionHypertension HematuriaHematuria Azot
16、emiaAzotemia Injures of the capillary Injures of the capillary wallwallGFR decreaseGFR decreaseFluid Fluid retentionretentionFluid retention,renin Fluid retention,renin increaseincreasewaste products increasewaste products increaseglomerular damage glomerular damage Crescentic Crescentic glomerulone
17、phritisglomerulonephritisCresentic Cresentic glomerulonephritisglomerulonephritisCresentic Cresentic glomerulonephritisglomerulonephritisCrescentic Crescentic glomerulonephritisglomerulonephritisCrescentCrescent Basement membrane Basement membrane destructiondestruction Cresentic Cresentic glomerulo
18、nephritiglomerulonephritis sGoodpasture syndromeGoodpasture syndromenMembranous glomerulopathyMembranous glomerulopathyMembranous glomerulopathyMembranous glomerulopathyMembranous glomerulopathyMembranous glomerulopathyIgG IgG Immunofluorescence Immunofluorescence Clinical ManifestationsClinical Man
19、ifestationsnMinimal change Minimal change diseasediseaseFSGSFSGSFSGSFSGSFSGSFSGSIgM IFIgM IFnMembranoproliferative glomerulonephritisMembranoproliferative glomerulonephritisIgGIgG IFIFnPrognosis is poorPrognosis is poornChronic glomerulonephritisChronic glomerulonephritisIgA nephropathyIgA nephropat
20、hy (PAS)(PAS)IgA nephropathy(PASM)IgA nephropathy(PASM)IgA Immunofluorescence IgA Immunofluorescence IgA Immunofluorescence IgA Immunofluorescence nChronic glomerulonephritisChronic glomerulonephritistubular atrophy and interstitial tubular atrophy and interstitial fibrosisfibrosisChronic glomerulon
21、ephritisChronic glomerulonephritisChronic renal Chronic renal failurefailureEnd stage kidneyEnd stage kidneyHypertensioHypertension n Proteinuria Proteinuria AnemiaAnemiaAzotemiaAzotemiaUremiaUremia Edema Edema Uremic pericarditisUremic pericarditis“Cor “Cor villosum”villosum”Acute diffuse Acute dif
22、fuse proliferative GNproliferative GNacute nephritic syndromeacute nephritic syndromeRoute of infectionRoute of infectionHematogenous infectionHematogenous infection(descending infection)(descending infection)Ascending infectionAscending infectionAcute pyelonephritisAcute pyelonephritisPapillary Pap
23、illary necrosisnecrosisGross featureGross feature not equally damagednot equally damaged Irregular areas of Irregular areas of scarringscarring Marked calyceal Marked calyceal deformitiesdeformitiesChronic Chronic pyelonephritispyelonephritisChronic Chronic pyelonephritispyelonephritis not equally d
24、amagednot equally damaged Irregular areas of Irregular areas of scarringscarring Marked calyceal Marked calyceal deformitiesdeformitiesChronic pyelonephritisChronic pyelonephritis“Thyroidization”“Thyroidization”Dilation of Dilation of tubulestubules急性肾小管坏死急性肾小管坏死(Acute tubular(Acute tubular necrosis
25、)necrosis)急性肾小管坏死急性肾小管坏死过敏性间质性肾炎过敏性间质性肾炎过敏性间质性肾炎过敏性间质性肾炎Renal calculusRenal calculusRenal staghorn calculusRenal staghorn calculusS SFluoroscopyFluoroscopy Ureteral Ureteral calculuscalculusHydronephrosisHydronephrosis Normal Normal urinary urinary tracttractIntravenous Intravenous pyelogram(IVP)pye
26、logram(IVP)HydronephrosisHydronephrosis Intravenous Intravenous pyelogram(IVP)pyelogram(IVP)HydronephrosisHydronephrosis(cut surface)(cut surface)Staghorn Staghorn calculuscalculusHydronephrosis Hydronephrosis AbscessAbscess CTCTRenal cell carcinomaRenal cell carcinomaRenal cell carcinomaRenal cell
27、carcinomaRenal cell carcinoma invade into the renal Renal cell carcinoma invade into the renal veinvein Renal cell carcinoma Renal cell carcinoma(clear cell type)(clear cell type)Renal cell carcinoma(clear cell Renal cell carcinoma(clear cell type)type)Papillary renal cell carcinomaPapillary renal c
28、ell carcinomachromophobe renal cell chromophobe renal cell carcinomacarcinomaWilms tumor Wilms tumor(Nephroblastoma)(Nephroblastoma)Common malignant Common malignant tumors of childhoodtumors of childhood Abdominal Abdominal enlargementenlargementGenetic defects Genetic defects on chromosome 11on ch
29、romosome 11Wilms tumorWilms tumorAngiomyolipoma Angiomyolipoma Angiomyolipoma Angiomyolipoma Metastatic carcinoma of the Metastatic carcinoma of the kidneykidneyUrothelial carcinoma of the bladderUrothelial carcinoma of the bladderUrothelial carcinoma of the ureterUrothelial carcinoma of the ureterI
30、ntravenous Intravenous pyelogram(IVP)pyelogram(IVP)Carcinoma of Carcinoma of the bladderthe bladderCarcinoma of renal Carcinoma of renal pelvispelvisUrothelial carcinoma of pelvisUrothelial carcinoma of pelvisninvasive urothelial carcinoma,high invasive urothelial carcinoma,high gradegradegood recov
31、ery.good recovery.The most likely The most likely diagnosis is renal cell diagnosis is renal cell carcinomacarcinomaThe most common pattern is clear cell The most common pattern is clear cell type of RCC.The cause is of lipid and type of RCC.The cause is of lipid and glycogen within the cytoplasm of
32、 glycogen within the cytoplasm of neoplastic cellsneoplastic cellsThe size of the tumor,the The size of the tumor,the perinephrotic infiltration,the perinephrotic infiltration,the involvement of lymph node and the involvement of lymph node and the involvement of vessels.If the involvement of vessels
33、.If the tumor is confined within the renal tumor is confined within the renal capsule,there is a 70%10-year capsule,there is a 70%10-year survival.survival.ProstateProstatenSigns and symptoms of urinary tract Signs and symptoms of urinary tract obstructionobstructionnDysuria,urinary frequency,noctur
34、ia Dysuria,urinary frequency,nocturia increasedincreasednUrinary retention,bladder expansionUrinary retention,bladder expansionnUrinary tract infections,hydronephrosisUrinary tract infections,hydronephrosis obstruction with bladder hypertrophy and formation of the stoneProstate carcinoma Prostate carcinoma with bone with bone metastasismetastasisMature teratomaMature teratomaMature Mature teratomateratoma(Dermoid cyst)(Dermoid cyst)Mature teratomaMature teratomaImmature teratomaImmature teratomaSuggested readingSuggested reading