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Bilingualcasediscussion2015-09-11
1A35-year-oldmanwithdyspnea,anemia,andrenalfailure
2severalweeksago,thepatientfeltfatigue.Twodaysbeforeadmission,dyspneadeveloped,decreasedurination,withoutfever,cough,orchestpain,nohemoptysis咯血,chills,nightsweats,headaches,visualloss,dryeyes,drymouth,orjointpain.Hewastransferredbyambulancetotheemergencydepartmentatthishospital
3Fouryearsbeforeadmission,hehadbeenevaluatedbecauseofbackpain,fatigue,andatemperatureof38.6°C.Urinalysisshowedhematuria血尿andproteinuria；testingforrapidplasmareagin(RPR快速血浆反应素)waspositiveforantibodiestoTreponemapallidum（梅毒螺旋体）.Duringtheprevious6months,thepatienthadhadepisodesofbilateralfinger,ankle,andfacialswelling,withoutpainorchangeincolor.Hehadnohistoryofrecenttravel,exposuretosickpersons,bloodtransfusions,orprevioussurgery.Hedidnotsmoke,drinkalcohol,oruseillicitdrugs.
4Onexamination,T36.9°C,BP173/89mmHg,P95b/m,R36b/m,andS0288%(ambientair).Theskinandconjunctivae结膜werepale,andtherewerehypopigmentedmacules色素减退斑ontherighttempleandbothlowercheeksandhyperpigmentedmacules色素沉着斑onthebridgeofthenose.Therewerebibasilarrales双肺底湿罗音inthelungs,andtheremainderoftheexaminationwasnormal.
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8totalanddirectbilirubin胆红素,liver-functiontestsandlactatewerenormal.testingforrheumatoidfactor,screeningofthebloodandurinefortoxinswerenegative;TestingforhepatitisBandCvirusesandautoantibodiesagainsthistones组蛋白wasnegative.Serumproteinelectrophoresis电泳revealedadiffuseincreaseintheIgGlevel.
9Transthoraciccardiacultrasonographyrevealednormalglobalcardiacfunctionandright-ventricularsize,noevidenceofapericardialeffusion心包积液,andfindingsthatwereconsistentwithpulmonaryEdema.Ultrasonographyoftheabdomenrevealednormalrenalsize,position,andechotexture回声特性andnormalarterialbloodflow.Anelectrocardiogram(ECG)showedsinustachycardia,counterclockwiserotation逆钟向,andnonspecificST-segmentandT-waveabnormalities.
10onadmission
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12ground-glassopacitiesGGO
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14Bronchoscopicexaminationrevealedthick,redmucusinthemain-stemandright-lower-lobebronchi;airwaysoftheleftlungwerenormal.Bronchoalveolarlavageontheright,with300and24,500redcellspercubicmillimeter(inthefirsttube),975and1475whitecellspercubicmillimeter(infourthtubes)Inthefourthtube,thewhite-celldifferentialcountrevealed84%leukocytes白细胞.Onthesecondday,thesputumculturegrewveryfewklebsiella克雷伯
15whatdoyouthink
16Pulmonaryhemorrhage？
17PCP卡氏肺孢子？（梅毒阳性、HIV）pulmonaryembolism肺栓塞？
18RapidlyprogressiveglomerulonephritisPulmonaryhemorrhage
19ErnestW.Goodpasturereportedin1919ontheautopsyfindingsinthecaseofan18-year-oldmanwhohaddiedofmassivelunghemorrhageandcrescenticglomerulonephritisduringtheheightoftheinfluenzapandemicThetermGoodpasture’ssyndromeisappliedtothecombinationoflungpurpuraandnephritis,regardlessoftheunderlyingpathogenesis.
20(一)anti-GBMdiseaseanti–glomerularbasementmembrane(GBM)antibodies(anti-GBMdisease)anti-GBMantibody
21（二）vasculitisvasculitis大血管炎大动脉炎巨细胞动脉炎中等血管炎结节性多动脉炎川崎病小血管炎ANCA相关性血管炎显微镜下型多血管炎（MPA）肉芽肿性多血管炎（GPA，Wegener’s）嗜酸细胞性肉芽肿性多血管炎（EGPA，CSS）免疫复合物相关性小血管炎冷球蛋白血管炎IgA血管炎低补体荨麻疹性血管炎（抗C1q血管炎）
22（三）infectionAvarietyofbothsystemicandpulmonarymicrobialinfectionscanbeaccompaniedbypulmonaryhemorrhageandrenaldisease,includingnephritis.Inoneexceptionalcase,legionnaires’disease军团菌病.Noinfectionscouldbeimplicatedinthispatient.
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24Theantinuclearantibodytiterwaspositiveat1:1280andhadahomogeneouspattern.Thetiterforantibodiestodouble-strandedDNAwaspositiveat1:80.
25clinical+imaging+pathology
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27肾小体结构模式图
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29*足细胞（podocyte）有初、次级突起，其次级突起间相互嵌合为栅栏状，之间有裂孔，上有裂孔膜。
30③滤过膜filtrationmembrane又称滤过屏障filtrationbarrier，由有孔毛细血管内皮、基膜和足细胞裂孔膜构成。
31滤过屏障模式图返回
32diffuselythickenedcapillary wallsandmildendocapillaryproliferationCellularcrescents
33(arrows)tubularatrophyandinterstitialinflammationfragmentedredcellsTheGBMwasmarkedlythickened,impartinga“wireloop”appearance白金耳
34abundantgranularimmune-complexdepositioninthemesangiumandalongtheglomerularbasementmembraneinaclassic“fullhouse”pattern满堂亮(anti-IgGimmunofluorescence)Immunecomplexdepositionisalsoseenalongthetubularbasementmembrane
35abundantelectron-densedepositsareseeninamesangial,intramembranous,subepithelial,andsubendothelialdistribution
36showedstrongstainingwithIgG,IgM,IgA,C3,C1q,andkappaandlambdalightchainsinagranularpatterninthemesangiumandalongtheGBM
37所涉及的病理学术语的定义弥漫性病变(diffuse)：病变累及50％肾小球。局灶性病变(focal)：病变仅累及≤50％肾小球。球性病变(global)：病变累及一个肾小球的大部分毛细血管袢(>50％)。节段性病变(segmental)：病变仅累及一个肾小球的少部分毛细血管袢(≤50％)。系膜细胞增生(mesangialhypercellularity)：3μm切片中，一个系膜区超过3个细胞。毛细血管内增生(endocapillaryproliferation)：肾小球毛细血管内皮细胞和系膜细胞增生，单个核细胞浸润，导致毛细血管腔狭窄。
38I型轻微病变性狼疮性肾炎II型系膜增殖性狼疮性肾炎III型局灶性狼疮性肾炎III（A）：活动性病变—局灶增殖性LNIII（A/C）：活动和慢性化病变—局灶增殖和硬化性LNIII（C）：慢性非活动性病变—局灶硬化性LNIV型弥漫性狼疮性肾炎IV-S（A）IV-G（A）IV-S（A/C）Ⅴ型膜性狼疮性肾炎Ⅵ型终末硬化性狼疮性肾炎狼疮性肾炎的病理组织学分类（ISN/RPS2003）IV-G（A/C）IV-S（C）IV-G（C）
39根据活动性和硬化性病变又分为多个亚型IV-S（A）：活动性病变—弥漫节段增殖性LNIV-G（A）：活动性病变—弥漫球性增殖性LNIV-S（A/C）：活动和慢性化病变并存—弥漫节段增殖和硬化性LNIV-G（A/C）：活动和慢性化病变并存—弥漫球性增殖和硬化性LNIV-S（C）：慢性非活动性病变伴疤痕形成—弥漫节段硬化性LNIV-G（C）：慢性非活动性病变伴疤痕形成—弥漫球性硬化性LNIV型弥漫性狼疮性肾炎
40SLEwithdiffuseproliferativelupusnephritis,withactiveandchroniclesions(classIV-G[A/C]),andpulmonaryalveolarhemorrhage.
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43梅毒检测临床上通常用快速血浆反应素环状卡片试验(RPR)筛查梅毒患者。梅毒螺旋体在破坏组织时，释放出心磷脂．刺激机体产生抗心磷脂抗体。RPR用从牛心提取的心磷脂加卵磷脂和胆固醇的组合成分作为抗原。与机体产生的反应素在体外反应。而牛心提取的心磷脂可与患者血清中抗心磷脂抗体发生免疫反应。CTD患者体内同样存在抗心磷脂抗体，因此RPR在CTD中存在假阳性，尤其是SLE、APS。国外报道SLE患者中25％一30％出现RPR假阳性
44Takehomemassages1.梅毒假阳性原因2.Goodpasture’ssyndrome3.狼疮肾炎
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