八制自身免疫课件

教学目的与要求了解:病理分期（渗出、增生、硬化期）熟悉:

病因;

发病机理;

鉴别诊断掌握:

临床表现;

Jones诊断标准；

治疗预防原则;风湿热活动指标教学目的与要求了解:病理分期（渗出、增生、硬化期）RheumaticfeverisanimmunologicalinflammatorydiseasefollowsinfectionwithcertainstrainsofgroupAstreptococcieasilyrecurwithoutprophylaxiscarditischoreamigratorypolyarthritissubcutaneousnodulespermanentvalvulardiseaseerythemamarginatumRheumaticfeverisanimmunolo

Epidemiology

incidence：22/100000

inChina

season：winterorspringage：5–15yEpidemiology北京儿童医院1477名风湿热住院患者年龄分布约90%患者为>7岁儿童北京儿童医院1477名风湿热住院患者年龄分布约90%患者为>Etiology☆

anonsuppurativecomplicationofgroupAstreptococcalinfectionoftheupperrespiratorytract☆

occurs1-4weeksafterconvalescenceofinfection☆

individualpropensity☆

environmentalfactorslatitudealtitudehumiditynutritioncrowdingageEtiologylatitudealtitudePathogenesismolecularmimicryofbacterialantigenssimilaritybetweenbacterialandselfmoleculesasrecognizedbyimmunecellsleadingtoacross-reactwithtargetorgansinthebodycirculatingimmunecomplexes(CIC)

circulatingimmunecomplexesactivatethecomplementsystemleadingtotheinflammatorychangesGeneticpronenessHLA-B35、HLA-DR4Pathogenesismolecularmimicrycapsule（synovialmembranes）Cellwallprotein（myocardium,endocardium）Cellwallpolysaccharides（myocardium,endocardium）

cellmembraneprotein

（myocardium

、subthalamicnucleus、caudatenucleus）TheantigensofGroupAstreptococciandmolecularmimicrycapsule（synovialmembranes）Cel

pathology

急性渗出期（acuteexudativeperiod）增生期（proliferativeperiod）硬化期(scleroticperiod)

1

month3~4months2~3monthsconnectivetissueedemas,effuse,anddegenerate,infiltratedwithinflammatorycells.

Aschoffbodyinmyocardium,muscle,endocardium,subcutaneoustissuecollagenfiberhyperplasiaandscartissueformationmitral>aortic>tricuspid>pulmonarypathology1month3~acuteexudativeperiodedemaanddegenerationofcollagenandexudation

inpericardiumpericardialeffusionfibrinouspericarditisacuteexudativeperiodedemaanproliferativeperiod

Aschoffbodyinendocardium中心：fibrinoidnecrosisofcollagen

外周：lymphocytes,plasmacellsandAschoffgiantcellsAschoffgiantcelllargecellswithtwoormorepalenucleithathaveprominentnucleoli.

proliferativeperiodAschscleroticperiodmitralvalveshowsthickeningdistortedcusps,adherentcommissureswithcalcificationandthrombusdeposition,fusionandshorteningofchordaetendinae.stenoticmitralvalveshowsfusionofcommissures,missuresarefused;cuspsareseverelythickened.Thevalveisbothincompetentandstenotic.scleroticperiodmitralvalvesClinicalManifestationMajorclinicalmanifestations:

carditis;polyarthritis;chorea;subcutancousnodules;erythemamarginatumOrdinarycomplaints:

fever/arthralgia

Durationofacuterheumaticfever:

≤6monthsClinicalManifestationMajorcl

rheumaticcarditisIncidence:

40~50%OneandonlypermanentdamageEndocarditisMyocarditisPericarditis

Congestiveheartfailureduringtheinitialepisode:

5%~10%Pancarditis

rheumaticcarditisIncidence:MyocarditisTachycardiadisproportionatetothefeverCongestiveheartfailureGalloprhythmSoftsystolicmurmurheardattheapexECGabnormalitis：arrhythmias;prolongationoftheP-Rinterval;atrioventricularblock(AVB)

Cardiomegalyonx-rayBeforetreatment

aftertreatment

MyocarditisTachycardiadisprop

EndocarditisMitralregurgitation:

ApicalsystolicmurmurattheapexRelativemitralstenosis:

Low-pitchedmid-diastolicrumbleAorticregurgitation:

Diastolicmurmurinthethirdcostaattheleftsideofthesternum

EndocarditisMitralregurgitat15

PericarditisPrecordialpainPericardialeffusion

Africtionrub

pericardialtamponade

hypotension;muffledheartsounds;jugularvenousdistensionStrikingincreaseinheartsizeonX-rayEchocardiography:pericardialeffusion＞50ml

PericarditisPrecordialpainRheumaticarthritisIncidence:

50%~60%

Acutemigratorypolyarthritis

Largerjointsoftheextremitiesareaffected:

knee、ankle、elbow、wrist

Red,hot,swollen,exquisitelytender

andpainfulifmoved

asonejointrecovered,anotherjointmaybeinvolved

arthritislastslessthan1monthwithoutdeformityRheumaticarthritisIncidence:ChoreaIncidence:3%~10%Female＞male;8~12

yeasoldSudden,aimless,irregularmovementsoftheextremitiesandfacialmusclesthatsubsideduringsleepandexaggeratedbyemotionsEmotionalinstability:nervousMuscleweaknessandataxia:

clumsy,stumble,handwritingorspeechdisordersChoreaIncidence:3%~10%erythemamarginatum

Thecharacteristicrashesconsistofanevanescent,pink,erythematousmaculae,withaclearcenterandserpiginousoutline.Therashistransient,migratoryandnonpruritic,whichfoundprimarilyonthetrunkandproximalextremities.erythemamarginatum

Thechsubcutaneousnodules

Subcutaneousnodulesarepainlesssmallswellings

overbonyprominences,primarilyovertheextensortendonsofthehands,feet,elbows,scalp,scapulae,andvertebrae.Nodulestendtooccurincropsandmaypersistfordaystomonthsaftertheonsetofacuterheumaticfever.subcutaneousnodules

OtherclinicalfeaturesVariablefeverTirednesspalenessPneumoniaNosebleedsweatingAbdominalanginaOtherclinicalfeaturesVariablLaboratoryfindingsBloodroutinetest:WBC↑,mildanemiaAcutephasereactants：ESR↑,CRP↑IsolationofgroupAstreptococci(+)Serumantibodyagainstthespecificstrptococci:ASO↑,ASK↑,AH↑,anti-DNaseB↑Immunesystem:IgG↑,IgA↑,C3

↑ECG:P-Rinterval↑,seconddegreeAVBRoutineroentgenogramEchocardiographyLaboratoryfindingsBloodrouti

TheJonesCriteriaRevisedwithAdditionofWorldHealthOrganizationRecommendationsMajorCriteriaMinorCriteriaCarditisFeverPolyarthritis,migratoryArthralgiaErythemamarginatumincreasedacute-phasereactantsChoreaESR↑,CRP↑SubcutaneousnodulesProlongedP-Rinterval

PlusEvidenceofaprecedinggroupAstreptococcalinfection(culture,rapidantigen,antibodytitersrise/elevation,historyofscarletfever)★

twomajormanifestations+EvidenceofS.I(streptococcalinfection)★

onemajor＋twominormanifestations+EvidenceofS.ITheJonesCFever,bodyweight↓,tirenessTachycardiaorarrhythmiasESR↑,CRP↑,neutrocyte↑,antibodytiter↑DignosisofactiverheumaticfeverDignosisofactiverheumaticfDifferentialdiagnosis

Fever

Carditis

ArthritisDifferentialdiagnosisFeverDifferentialdiagnosisofcarditisInfectiveendocarditis：

anemia,splenomegaly,petechia,embolismbloodculture（+）vegetationsonendocardium/valvesViralmyocarditis：

arrhythmias(prematurecontraction)evidenceofviralinfection

DifferentialdiagnosisofcardDifferentialdiagnosisofarthritisSystemiclupuserythematosus(SLE):

malarrash,proteinuria,hypertension,leukopenia,Coombs(+)hemolyticanemia,antinuclearantibodies(+)Juvenilerheumatoidarthritis(JRA)：

morningstiffness,iridocyclitis,progressionofjointdestruction,ANA(+),rheumatoidfactor(+)DifferentialdiagnosisofarthBedrest

antibiotics

anti-rheumatismtherapy

heteropathyManagementBedrestManagement（1）Bedrest

carditiscardiamegalycongestiveheartfailure------2w2w+----4w4w++--6w6w+++8w3mon（1）Bedrestcarditiscardiamegal（2）antibioticsProcainepenicillinG:

4.8millonU~9.6millonU/d，ivdrip×2~3w

PG

AST(+):

Erythromycinp.o×10d（3）anti-rheumatismtherapyCarditis：Prednisone,2mg/kg.d(≤60mg/d)×2~4w;reducedosegradually;fullduration=8~12warthritis：Aspirin,80~100mg/kg.d(≤3g/d)untilremission;graduallyreducetohalfdosefor4~6w（2）antibiotics（4）heteropathy

congestiveheartfailure：steroid;oxygentherapy;diuresis;captopril；digitalis(smalldose)chorea：tranquilizer(chlorpromazine,barbital)

arthralgia:

immobilizationofaffectedjoints（4）heteropathyprophylaxis

Recurrentrheumaticfever

benzathinePenicillin:1.2millionU,Q4W,≥5yearspatientswithestablishedheartdiseasemaycontinuefor≥10years,eventhewholelife.PG

AST(+):

Erythromycinp.o×6~7d,everymonth

BacterialEndocarditis

PphylaxisRecurrentrheumatiEmphasesFivemajorclinicalmanifestationsJonescriteriaFeaturesofactiverheumaticfevertreatment：prophylaxis：long-actingPGEmphasesFivemajorclinicalmKawasakidisease（Mucocutaneouslymphnodesyndrome）川崎病Kawasakidisease（Mucocutaneous教学目的与要求了解:病因；病理分期熟悉:辅助检查；预后掌握:临床表现；诊断；

治疗原则教学目的与要求了解:病因；病理分期

TomisakuKawasakidescribedKDin1967

KDisaacutegeneralizedsystemicvasculitisofunknownetiologywithfeverandrashes.CoronaryarterydilationoraneurysmsKDhasreplacedacuterheumaticfeverasthemostcommoncauseofacquiredheartdiseaseinchildrenIndevelopedcountriesTomisakuKawasakidescribedKAge：<4

yearsold(80%)<2

yearsold(50%)Sex：moreofteninmalesthaninfemales(1.5:1)Season：clustersinwinter/spring

Racialbackground:Asianchildren,especiallythoseofJapanesedescent.EpidemiologyAge：<4yearsold(80%)EpidemEtiologyandPathogenesis

etiologyofKDremainsundiscovered.immunopathogenicmechanismforcoronarydiseaseorganismsuper-antigen

mimicantigen(HSP65)Tcell-mediatedimmuneresponsecytokine–mediatedimmunedamageEtiologyandPathogenesisetiostageⅠ：1~10d，acutesmallperiarteritis；cardiacinflammatorychangesstageⅡ：10~25

d，coronaryarteritis;elasticlaminaeandmuscularlayerssplit,leadingtothrombusandaneurysms.stageⅢ：26~31

d，acuteinflammationremission;fibroustissueproliferates;intimathickens;coronaryarteriesnarroworocclude.stageⅣ：≧40d,cicatrizationinmyocardium;

occludedarteriesreopen.Pathophysiology

—systemicvasculitis(coronaryarteries)stageⅠ：1~10d，acutesmallperinormal

coronaryartery

stageⅠstageⅡnormalcoronaryarterystageⅠs10daysaftertheonsetofsymptoms,elasticlaminaesplits,intimaproliferatesandthickensinbranchofcoronaryartery.

10daysaftertheonsetofsymHugecoronaryarteryaneurysmHugecoronaryarteryaneurysmClinicalmanifestation

Mucocutaneouslymphnodeabnormalities

Cardiovascularabnormalities

OthernonspecificallymanifestationsClinicalmanifestationMucocutMainclinicalfeatures1.Feverusuallymorethan39°C,foratleast5daysHighspikingandremittentnotrespondstoantibioticsGenerallypersists1-2weekswithouttreatmentusuallyresolvesin1-2daysaftertreatmentwithintravenousgammaglobulin(IVIG)Mainclinicalfeatures1.Feve2.BilateralconjunctivainjectionwithoutexudateMainclinicalfeaturesMainclinicalfeaturesMainclinicalfeatures3.inflammationofthelipsandoralcavityInjected,dry,fissured-lipsinjectedoralandpharyngealmucosaStrawberrytonguewithprominentpapillaeanderythemanooralexudates,ulcerations,orKoplikspotsMainclinicalfeatures3.inflaMainclinicalfeatures4.HandsandfeetErythema,orindurativeedemaofpalmsandsolesPeriungualmembranousdesquamationoffingersandtoesabout2weeksafteronsetTransversegroovesacrossthenailsMainclinicalfeatures4.HaMainclinicalfeatures5.rashofvariousformsdiffuse,scarlatiniformorerythemapolymorphousrasherythemaordesquamationinperinealregionMainclinicalfeatures5.Mainclinicalfeatures6.non-purulentcervicallymphadenopathy50-75%ofpatientsWithanodesizeof1.5cmorgreaterindiametertenderness,notredMainclinicalfeatures6.1.

carditisTachycardiaGalloprhythm

systolicmurmursArrhythmia2.myocardialischemia

anginamyocardialinfarctionCardiovascularabnormalities1.carditisCardiovascularabn3.Coronaryarterialchanges

—2~4weeksafteronset/convalescent

phase

coronaryarteritisvesselintimaroughened

coronaryarteriesnarrow

coronaryarteriesdilation

coronaryarteryaneurysm3.Coronaryarterialchanges

冠状动脉瘤（CoronaryArteryAneurysm）最早于发病第6天检出，8～12周明显急性期发生率最高为25～30%，恢复期发生率10～20%。急性期一过性冠状动脉扩大（46%）持续性冠状动脉瘤（21%）：多数1～2年内恢复，约5-6%不恢复。主要累及冠状动脉主干近端

冠状动脉瘤（CoronaryArteryAneurysAneurysmatleftanteriordescending(LAD)coronaryarteryLADCoronaryArteryAneurysm

—

20~30%ofuntreatedchildren冠状动脉瘤发生率：左前降支＞左冠状动脉主干、右冠状动脉＞左回旋支左回旋支Aneurysmatleftanteriordesc53HighriskfactorsofCAaneurysm

age:<6monthor>3yearsmalesexfeverformorethan16daysorrecurrencecardiomegalyorarrhythmialabfindings:

Hb<80g/L,WBC>16~30X109/L,PLT>1000X109/L,ESR>100mm/hKDrecurrenceHighriskfactorsofCAaneuryLess-commonfeatures

asepticmeningitisabdominalpainotitismediajaundicediarrhea

gallbladderhydropshepaticdysfunctionarthralgiaarthritisurethritisLess-commonfeaturesasepticgBloodanalysis：

WBC↑;mildanemia;PLT↑in2nd~3thweek；

ESR↑；

CRP↑；

ALT↑;AST↑

LaboratoryfindingsBloodanalysis：LaboratoryfinImmunesystem

IgG、IgM、IgA、IgE↑；

CirculatingImmuneComplexes

↑；C3

normalor↑ImmunesystemECG：ST-T

abnormalitiesofpericarditisormyocardialinfarction

非特异性ST-T变化；心律失常;心包炎时广泛ST段抬高、低电压；心肌梗死时ST段明显抬高、T波倒置、病理性Q波；Chestroentgenogram：nonspecificperihilarorparenchymainfiltrates；

cardiamegaly.ECG：ST-TabnormalitiesofperiEchocardiography

coronaryarteritisintimaroughened

coronaryarteriesnarrowordilation

coronaryarteryaneurysm

pericardialeffusion

mitral,aortic,ortricuspiddisturbedflowcoronaryarteryaneurysmrightcoronaryarterytrunkaortaEchocardiographycoronaryart

冠状动脉扩张：

冠状动脉内径>正常范围冠状动脉内径与主动脉根部内径之比>0.3

正常冠状动脉主干内径

0～3岁<2.5mm3～

9岁

<3mm9～

14岁<3.5mm

冠状动脉扩张的分级轻度3mm＜冠状A直径≤4mm

中度4mm＜冠状A直径≤7mm

重度冠状A直径≥8mm（冠状动脉瘤）

Coronaryangiography

—myocardialischemia/multiplecoronaryaneurysmsnormalaneurysmLADdilationandnarrowCoronaryangiographynormalaneuDiagnosticguidelines

(fortypicalcases)feverlastingmorethan5days+4ofthefollowing5criteria(otherillnessesmustbeexcluded):

1.polymorphousrash2.bilateralconjunctivalinjectionwithoutexudatediffuseinjectionoforalmucosa,erythemaorfissuringofthelips,strawberrytongue4.nonpurulentcervicallymphnodeenlargement(onelymphnode>1.5cm)5.extremitychanges:erythemaofpalms/soles,indurativeedemaofhands/feet,MembranousdesquamationofthefingertipsDiagnosticguidelines

(fortyDiagnosticguidelines

(foratypicalcases)feverlastingmorethan5days

≤3ofthe5criteria

coronaryarteriesdilationoraneurysmdetectedbyechocardiography

Diagnosticguidelines

(foratDifferentialdiagnosisScarletfever

Redrashblancheswithpressure,whichisdiffusebutsparesthepalms,soles,andface.Thefaceappearsflushed.Theskinrashfadesinaweekandisfollowedbyextensivedesquamation.PatienthasgoodresponsetoPG.DifferentialdiagnosisScarletDifferentialdiagnosisExudativeandErythemaMultiforme

polymorphousErythema,herpesandextensivedesquamation;oralulcers;conjunctivalexudate;noindurativeedemaofpalmsorsolesDifferentialdiagnosisExudativ

relievevasculitisinhibitPLTaggregationTreatment&MedicationrelievevasculitisTreatment（1）aspirin

administeredforanti-inflammatoryandantithromboticeffects

acutephase:30-100mg/kg/dPOintid/qid72hafterdefervescence:reducedosegradually2weeksafterdefervescence:3-5mg/kg/dp.o×6~8weeksuntilESR,PLTandcoronaryarteriesreturntonormal（Ø<3mm）（1）aspirinreducetheprevalenceofcoronaryabnormalitiesandleadtorapiddefervescence

免疫调节负反馈作用，减少IgG合成，封闭血管内皮细胞、单核-巨噬细胞和血小板表面的Fc受体，提供未知特异性抗体和抗毒素，降低冠状动脉病变发生。

2g/kgIVinfusionover8-12hwithin10dafteronsetPatientwithincompleteresponsecanreceiveasecondcourseofIVIGandcorticosteriods.

deferusingliveviralvaccinesuntilabout11monthsafterIVIGadministration(2)intravenous

gammaglobulin(IVIG)reducetheprevalenceofcoron

indicationsofadministration

pancarditisnoavailableIVIGnoresponsetoIVIGprednisone/methylprednisolone

combinationwithaspirin+persantine

(3)corticosteriodsindicationsofadministrationpersantine3~5mg/kg/dmaintenancetreatmentinpatientwithhugeormultiplecoronaryaneurysmsaspirin3~5mg/kg/d+warfarin(4)inhibitPLTaggregation(5)OthertherapyLiquidtherapythrombolyticdrugcoronaryarterybypassgraftpersantine3~5mg/kg/d(4)inhibPrognosis

principalcauseofdeath:

myocardialinfarction

fatalityrate：0.5-1%

recurrencerate:1-2%

incidenceofCAaneurysms:20~30%inuntreatedpatient15%inIVIGtreatedpatient

Prognosisprincipalcauseofoutpatientfollow-upsystemicexamination

physicalexaminationECG;Echocardiography

noCAabnormality:thefirst1,3,6,12

month

CAabnormality:thefirst1,3,6

month,then

every6-12

monthuntilCAreturntonormaloutpatientfollow-upsystemicEmphasesacutegeneralizedsystemicvasculitiscoronaryarteryabnormalitiesdiagnosticguidelinestreatment：goals/medicationoutpatientfollow-upEmphasesacutegeneralizedsysM(y)Heart

M:mucocutaneousH:hand

/feetE(eye)：conjunctivaA:adenopathy(lymphadenopathy)R:rashT（tempreture）：fever

M(y)Heart

比较风湿热、川崎病心脏损害的特点与治疗原则。课后复习课后复习THANKYOU!THANKYOU!教学目的与要求了解:病理分期（渗出、增生、硬化期）熟悉:

病因;

发病机理;

鉴别诊断掌握:

临床表现;

Jones诊断标准；

治疗预防原则;风湿热活动指标教学目的与要求了解:病理分期（渗出、增生、硬化期）RheumaticfeverisanimmunologicalinflammatorydiseasefollowsinfectionwithcertainstrainsofgroupAstreptococcieasilyrecurwithoutprophylaxiscarditischoreamigratorypolyarthritissubcutaneousnodulespermanentvalvulardiseaseerythemamarginatumRheumaticfeverisanimmunolo

Epidemiology

incidence：22/100000

inChina

season：winterorspringage：5–15yEpidemiology北京儿童医院1477名风湿热住院患者年龄分布约90%患者为>7岁儿童北京儿童医院1477名风湿热住院患者年龄分布约90%患者为>Etiology☆

anonsuppurativecomplicationofgroupAstreptococcalinfectionoftheupperrespiratorytract☆

occurs1-4weeksafterconvalescenceofinfection☆

individualpropensity☆

environmentalfactorslatitudealtitudehumiditynutritioncrowdingageEtiologylatitudealtitudePathogenesismolecularmimicryofbacterialantigenssimilaritybetweenbacterialandselfmoleculesasrecognizedbyimmunecellsleadingtoacross-reactwithtargetorgansinthebodycirculatingimmunecomplexes(CIC)

circulatingimmunecomplexesactivatethecomplementsystemleadingtotheinflammatorychangesGeneticpronenessHLA-B35、HLA-DR4Pathogenesismolecularmimicrycapsule（synovialmembranes）Cellwallprotein（myocardium,endocardium）Cellwallpolysaccharides（myocardium,endocardium）

cellmembraneprotein

（myocardium

、subthalamicnucleus、caudatenucleus）TheantigensofGroupAstreptococciandmolecularmimicrycapsule（synovialmembranes）Cel

pathology

急性渗出期（acuteexudativeperiod）增生期（proliferativeperiod）硬化期(scleroticperiod)

1

month3~4months2~3monthsconnectivetissueedemas,effuse,anddegenerate,infiltratedwithinflammatorycells.

Aschoffbodyinmyocardium,muscle,endocardium,subcutaneoustissuecollagenfiberhyperplasiaandscartissueformationmitral>aortic>tricuspid>pulmonarypathology1month3~acuteexudativeperiodedemaanddegenerationofcollagenandexudation

inpericardiumpericardialeffusionfibrinouspericarditisacuteexudativeperiodedemaanproliferativeperiod

Aschoffbodyinendocardium中心：fibrinoidnecrosisofcollagen

外周：lymphocytes,plasmacellsandAschoffgiantcellsAschoffgiantcelllargecellswithtwoormorepalenucleithathaveprominentnucleoli.

proliferativeperiodAschscleroticperiodmitralvalveshowsthickeningdistortedcusps,adherentcommissureswithcalcificationandthrombusdeposition,fusionandshorteningofchordaetendinae.stenoticmitralvalveshowsfusionofcommissures,missuresarefused;cuspsareseverelythickened.Thevalveisbothincompetentandstenotic.scleroticperiodmitralvalvesClinicalManifestationMajorclinicalmanifestations:

carditis;polyarthritis;chorea;subcutancousnodules;erythemamarginatumOrdinarycomplaints:

fever/arthralgia

Durationofacuterheumaticfever:

≤6monthsClinicalManifestationMajorcl

rheumaticcarditisIncidence:

40~50%OneandonlypermanentdamageEndocarditisMyocarditisPericarditis

Congestiveheartfailureduringtheinitialepisode:

5%~10%Pancarditis

rheumaticcarditisIncidence:MyocarditisTachycardiadisproportionatetothefeverCongestiveheartfailureGalloprhythmSoftsystolicmurmurheardattheapexECGabnormalitis：arrhythmias;prolongationoftheP-Rinterval;atrioventricularblock(AVB)

Cardiomegalyonx-rayBeforetreatment

aftertreatment

MyocarditisTachycardiadisprop

EndocarditisMitralregurgitation:

ApicalsystolicmurmurattheapexRelativemitralstenosis:

Low-pitchedmid-diastolicrumbleAorticregurgitation:

Diastolicmurmurinthethirdcostaattheleftsideofthesternum

EndocarditisMitralregurgitat91

PericarditisPrecordialpainPericardialeffusion

Africtionrub

pericardialtamponade

hypotension;muffledheartsounds;jugularvenousdistensionStrikingincreaseinheartsizeonX-rayEchocardiography:pericardialeffusion＞50ml

PericarditisPrecordialpainRheumaticarthritisIncidence:

50%~60%

Acutemigratorypolyarthritis

Largerjointsoftheextremitiesareaffected:

knee、ankle、elbow、wrist

Red,hot,swollen,exquisitelytender

andpainfulifmoved

asonejointrecovered,anotherjointmaybeinvolved

arthritislastslessthan1monthwithoutdeformityRheumaticarthritisIncidence:ChoreaIncidence:3%~10%Female＞male;8~12

yeasoldSudden,aimless,irregularmovementsoftheextremitiesandfacialmusclesthatsubsideduringsleepandexaggeratedbyemotionsEmotionalinstability:nervousMuscleweaknessandataxia:

clumsy,stumble,handwritingorspeechdisordersChoreaIncidence:3%~10%erythemamarginatum

Thecharacteristicrashesconsistofanevanescent,pink,erythematousmaculae,withaclearcenterandserpiginousoutline.Therashistransient,migratoryandnonpruritic,whichfoundprimarilyonthetrunkandproximalextremities.erythemamarginatum

Thechsubcutaneousnodules

Subcutaneousnodulesarepainlesssmallswellings

overbonyprominences,primarilyovertheextensortendonsofthehands,feet,elbows,scalp,scapulae,andvertebrae.Nodulestendtooccurincropsandmaypersistfordaystomonthsaftertheonsetofacuterheumaticfever.subcutaneousnodules

OtherclinicalfeaturesVariablefeverTirednesspalenessPneumoniaNosebleedsweatingAbdominalanginaOtherclinicalfeaturesVariablLaboratoryfindingsBloodroutinetest:WBC↑,mildanemiaAcutephasereactants：ESR↑,CRP↑IsolationofgroupAstreptococci(+)Serumantibodyagainstthespecificstrptococci:ASO↑,ASK↑,AH↑,anti-DNaseB↑Immunesystem:IgG↑,IgA↑,C3

↑ECG:P-Rinterval↑,seconddegreeAVBRoutineroentgenogramEchocardiographyLaboratoryfindingsBloodrouti

TheJonesCriteriaRevisedwithAdditionofWorldHealthOrganizationRecommendationsMajorCriteriaMinorCriteriaCarditisFeverPolyarthritis,migratoryArthralgiaErythemamarginatumincreasedacute-phasereactantsChoreaESR↑,CRP↑Subc