八制自身免疫課件

教學(xué)目的與要求了解:病理分期（滲出、增生、硬化期）熟悉:

病因;

發(fā)病機(jī)理;

鑒別診斷掌握:

臨床表現(xiàn);

Jones診斷標(biāo)準(zhǔn)；

治療預(yù)防原則;風(fēng)濕熱活動(dòng)指標(biāo)教學(xué)目的與要求了解:病理分期（滲出、增生、硬化期）RheumaticfeverisanimmunologicalinflammatorydiseasefollowsinfectionwithcertainstrainsofgroupAstreptococcieasilyrecurwithoutprophylaxiscarditischoreamigratorypolyarthritissubcutaneousnodulespermanentvalvulardiseaseerythemamarginatumRheumaticfeverisanimmunolo

Epidemiology

incidence：22/100000

inChina

season：winterorspringage：5–15yEpidemiology北京兒童醫(yī)院1477名風(fēng)濕熱住院患者年齡分布約90%患者為>7歲兒童北京兒童醫(yī)院1477名風(fēng)濕熱住院患者年齡分布約90%患者為>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

急性滲出期（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教學(xué)目的與要求了解:病因；病理分期熟悉:輔助檢查；預(yù)后掌握:臨床表現(xiàn)；診斷；

治療原則教學(xué)目的與要求了解: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

冠狀動(dòng)脈瘤（CoronaryArteryAneurysm）最早于發(fā)病第6天檢出，8～12周明顯急性期發(fā)生率最高為25～30%，恢復(fù)期發(fā)生率10～20%。急性期一過(guò)性冠狀動(dòng)脈擴(kuò)大（46%）持續(xù)性冠狀動(dòng)脈瘤（21%）：多數(shù)1～2年內(nèi)恢復(fù)，約5-6%不恢復(fù)。主要累及冠狀動(dòng)脈主干近端

冠狀動(dòng)脈瘤（CoronaryArteryAneurysAneurysmatleftanteriordescending(LAD)coronaryarteryLADCoronaryArteryAneurysm

—

20~30%ofuntreatedchildren冠狀動(dòng)脈瘤發(fā)生率：左前降支＞左冠狀動(dòng)脈主干、右冠狀動(dòng)脈＞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

非特異性ST-T變化；心律失常;心包炎時(shí)廣泛ST段抬高、低電壓；心肌梗死時(shí)ST段明顯抬高、T波倒置、病理性Q波；Chestroentgenogram：nonspecificperihilarorparenchymainfiltrates；

cardiamegaly.ECG：ST-TabnormalitiesofperiEchocardiography

coronaryarteritisintimaroughened

coronaryarteriesnarrowordilation

coronaryarteryaneurysm

pericardialeffusion

mitral,aortic,ortricuspiddisturbedflowcoronaryarteryaneurysmrightcoronaryarterytrunkaortaEchocardiographycoronaryart

冠狀動(dòng)脈擴(kuò)張：

冠狀動(dòng)脈內(nèi)徑>正常范圍冠狀動(dòng)脈內(nèi)徑與主動(dòng)脈根部?jī)?nèi)徑之比>0.3

正常冠狀動(dòng)脈主干內(nèi)徑

0～3歲<2.5mm3～

9歲

<3mm9～

14歲<3.5mm

冠狀動(dòng)脈擴(kuò)張的分級(jí)輕度3mm＜冠狀A(yù)直徑≤4mm

中度4mm＜冠狀A(yù)直徑≤7mm

重度冠狀A(yù)直徑≥8mm（冠狀動(dòng)脈瘤）

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（?<3mm）（1）aspirinreducetheprevalenceofcoronaryabnormalitiesandleadtorapiddefervescence

免疫調(diào)節(jié)負(fù)反饋?zhàn)饔?，減少IgG合成，封閉血管內(nèi)皮細(xì)胞、單核-巨噬細(xì)胞和血小板表面的Fc受體，提供未知特異性抗體和抗毒素，降低冠狀動(dòng)脈病變發(fā)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

比較風(fēng)濕熱、川崎病心臟損害的特點(diǎn)與治療原則。課后復(fù)習(xí)課后復(fù)習(xí)THANKYOU!THANKYOU!教學(xué)目的與要求了解:病理分期（滲出、增生、硬化期）熟悉:

病因;

發(fā)病機(jī)理;

鑒別診斷掌握:

臨床表現(xiàn);

Jones診斷標(biāo)準(zhǔn)；

治療預(yù)防原則;風(fēng)濕熱活動(dòng)指標(biāo)教學(xué)目的與要求了解:病理分期（滲出、增生、硬化期）RheumaticfeverisanimmunologicalinflammatorydiseasefollowsinfectionwithcertainstrainsofgroupAstreptococcieasilyrecurwithoutprophylaxiscarditischoreamigratorypolyarthritissubcutaneousnodulespermanentvalvulardiseaseerythemamarginatumRheumaticfeverisanimmunolo

Epidemiology

incidence：22/100000

inChina

season：winterorspringage：5–15yEpidemiology北京兒童醫(yī)院1477名風(fēng)濕熱住院患者年齡分布約90%患者為>7歲兒童北京兒童醫(yī)院1477名風(fēng)濕熱住院患者年齡分布約90%患者為>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

急性滲出期（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