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1、 风湿热风湿热 (Rheumatic fever)山东大学齐鲁医院儿科李福海 Summarization of rheumatic fevernDefinition:RF is an inflammatory disease which may develop after a Group A streptococcal infection(such as strep throat or scarlet fever)and can involve the heart,joints,skin,and brain.Post group A-hemolytic streptococcal infect
2、ion;(A组乙型溶链感染后)Connective tissue immunologic inflammatory disease (结缔组织免疫性炎性病变)Involving：carditis(心脏炎),polyarthritis(多发关节炎),chorea(舞蹈病),erythema marginatum(环形红斑),subcutaneous nodules(皮下结节).Summarization of rheumatic fevernIncidence:Related to purulent tonsillitis and scarlet;Applying penicillin in t
3、ime can prevent the development of rheumatic fever;Decreased in developed country;Countryside is more than city;Peak incidence is at the age between 6-15 y;Spring and winter;No sex difference.Etiology of rheumatic fever(RF)nGroupA-hemolyticstreptococcusinfectioncausedisthmitis(咽峡炎咽峡炎),then result in
4、 the autoimmunedisease.RF发发病病与与A组组B型型溶溶链链特特殊殊结结构构成成分分和和细细胞胞外外产产物有关物有关PathogenesisofRF nmolecularmimicryofstrep.antigen(链球菌抗原的分子模拟链球菌抗原的分子模拟):多种溶链的多种溶链的Ag与发病有关：与发病有关：荚膜透明质酸与人体关节、滑膜有共同荚膜透明质酸与人体关节、滑膜有共同Ag关节症状；关节症状；细细胞胞壁壁外外层层M蛋蛋白白和和M相相关关蛋蛋白白等等与与人人体体心心肌肌、心心瓣瓣膜膜糖蛋白有共同糖蛋白有共同Ag心脏症状；心脏症状；细细胞胞膜膜脂脂蛋蛋白白与与人人体体心心
5、肌肌纤纤维维膜膜和和丘丘脑脑下下核核、尾尾状状核核之之间有共同间有共同Ag中枢神经系统症状；中枢神经系统症状；Ag+Ab清除溶链清除溶链or器官损伤。器官损伤。PathogenesisofRFnimmunocomplexcausesdiseases.（免疫复合物致病）免疫复合物致病）StreptococcusAg+anti-StreptococcusAbCIC(circulatoryimmunocomplex)沉沉 积积 于于 joint synovium(关关 节节 滑滑 膜膜)、myocardiumorcardiacvalve(+)Complementinflammatoryreactio
6、nPathogenesisofRFncellularmediatedimmuneinjury(细胞免疫细胞免疫).Invitroreactionofpatientsperi-lymphocytetostrepAgwasenhanced；Cytotoxic effects of Patients monocyte onculturedmyocardiumcells;IncreasedNKcellcytotoxicity；AbnormalreactionofTonsilmonocytetostrepAg.PathogenesisofRFnhereditarypathogenesisnheredit
7、ary susceptibility nindividual difference of immune responsenRF suffers：Indian HLA-B35 American HLA-DR2 White people HLA-DR4 99%RF patients lymphocyte specific cell alloantigen(异抗原)is positive.（14）PathologyofRF nPathologicphaseslastingtimeExudativephase(渗出期渗出期)34weeksHyperplasiaphase(增生期增生期)34months
8、Sclerosisphase(硬化期硬化期)23monthsinvolvedorgans nexudationphase:cardio;jointsynovium;skin;nhyperplasiaphase:myocardiumandcardiacvalve(心肌、心瓣膜心肌、心瓣膜);pericardialmembrane(心外膜心外膜);epitenon(腱鞘腱鞘);Peripheralsubcutaneoustissue(关节周围皮下关节周围皮下T).nSclerosisphase:mitralvalve:stenosisorinsufficiencyaorticvalve:steno
9、sisorinsufficiencyPathologicfindingsExudationphase:degeneration;edema;inflammatorycellsinfiltration;fibrousexudation;hyperplasiaphase:AschoffsmallbodyorrheumaticgranulomaSclerosisphase:Scardevelopment;mitraloraorticvalvestenosisorinsufficiencyPathologicfindingsvMyocardial Aschoff body the cells are
10、large,elongated,with large nuclei;some are multinucleate clinicalmanifestationsofRFnSudden onset,carditis and chorea most express chronic course;nThe degree of clinical manifestations are differently;nLatent time:isthmitis,1to several weeks;nActive phase(no treatment):6m;nRecurrent onset(no prophyla
11、xis);clinicalmanifestationsofRF nCommonmanifestations:Fever;Fatigue;Pale;epistaxis(鼻出血鼻出血);abdominalpain;rheumaticpleurisy(风湿性胸膜炎风湿性胸膜炎);Pneumonia.clinicalmanifestationsofRFnArthritis:70%75multiplex:largejoints:redness,swelling,heat,severepain.migratory:Lasting：13w;Arthralgia(关节痛关节痛);Nojointdeformit
12、y.clinicalmanifestationsofRF nCarditis:40%50%myocarditis:Mildpatientshavenosymptoms;CHF(1015%):HR,heartsounddullness,gallop;cardiacdilation：LVenlarged;cardiacmurmur:systolic(mitral)ordiastolic(aorticvalve),23/6degree.EKG:P-Rinterval,Twavearrhythmia.度AV blockST-T changes of RFArrhythmia of RFclinical
13、manifestationsofRFEndocarditis:mitralinsufficiency:apex23/6pansystolicmurmur.aorticvalveinsufficiency:diastolicmurmur;mitralrelativestenosis:diastolicmurmur.v Stenotic mitral valve seen from left atrium.Both commissures are fused;the cusps are severely thickened.The left atrium is huge.The valve is
14、both incompetent and stenotic 心脏瓣膜病心脏瓣膜病vStenotic mitral valve seen from left atrium,showing fusion of commissures,thickening and calcification of the cusps clinicalmanifestationsofRFPericarditis:thoraxpain;pericardialfrictionrub;pericardialeffusion:chestX-rayorEchocardiogramcandiagnosis;pericardial
15、tamponade;EKG:lowervoltage,STsegment;Chest X-ray of RFSame patient after 4 weeksChest radiograph of an 8 year old patient with acute carditis before treatment 心包积液(pericardial effusion)左室后壁和心包之间左室后壁和心包之间可见液性暗区可见液性暗区betweenLVPWandpericardialcanseefluidarea(effusion).AVRAVR外所有常规导联中，外所有常规导联中，STST段弓背向下抬
16、高段弓背向下抬高心电图心电图clinical manifestations of RFnSydenhamchorea:515y,female;Extrapyramidal(锥体外系锥体外系)systemsigns:Psychologicmanifestations;mildpatientpersist34weeks;seriouspatientpersist34months；40%accompaniedwithcarditis.clinical manifestations of RFnSkinsymptoms:5%erythemamarginatum:环形、半环形边界清楚粉红色斑；环形、半环
17、形边界清楚粉红色斑；不痛、不痒；不痛、不痒；一分钱币大小，中心白，边缘高；一分钱币大小，中心白，边缘高；躯干、四肢近端屈侧；躯干、四肢近端屈侧；一过性，时隐时现。一过性，时隐时现。clinical manifestations of RFvErythema marginatum on the trunk,showing erythematous lesions with pale centers and rounded or serpiginous margins clinical manifestations of RFvCloser view of erythema marginatum
18、in the same patient clinical manifestations of RFnSkinsymptoms:subcutaneousnodules:圆形、硬、无痛结节；圆形、硬、无痛结节；粟米至黄豆大小；粟米至黄豆大小；与皮肤无粘连；与皮肤无粘连；见于肘、膝、腕、踝等关节伸面；见于肘、膝、腕、踝等关节伸面；24周消失。周消失。clinical manifestations of RF Subcutaneous nodules are rarely seen and when present,they are usually associated with severe car
19、ditis.They are painless,firm,movable,measuring around 0.5 to 2 cm.They are usually located over extensor surfaces of the joints,particularly knees,wrists and elbowslaboratoryexaminationofRF nTheproofsofstreptococcusinfection:throatswabculture:1/3streptococcusG+;serumantistreptolysinOtest:80%+;antide
20、oxyribonuclease(anti-DNase)BAb:95%+;Antistreptokinase(ASK,抗链球菌激酶抗链球菌激酶)Ab:95%+;anti-hyaluronidaseantibody:95%+.（抗透明质酸酶）抗透明质酸酶）laboratoryexaminationofRFnRheumaticfeveractiveindicators:WBC;Neutrophil;Anemia;ESR;CRP(+);2-globulin.Chorea以上指标为（）。以上指标为（）。DiagnosisofRF(Jones1992)nMajormanifestations(主要表现主要
21、表现):carditis;Polyarthritis;Sydenhamchorea;Erythemamarginatum;Subcutaneousnodules.DiagnosisofRFnMinormanifestations(次要表现次要表现):Fever;Arthralgia（关节痛）关节痛）;Postrheumaticfeverhistory;ESR、CRP+;P-Rintervalprolonged.Diagnosis of RFnProofsofstreptococcalinfections:Throatswabculture(+);Quicklystreptococcalanti
22、gen(+);ASOtiter；Recentscarletfeverhistory.Definitediagnosiscriteria:ntwomajorpresentations；nonemajorpresentationplustwominorpresentations；nbothhavetheproofsofstreptococcusinfection.Definitediagnosis:nCautions：Excludingotherdiseases；Carditisisthedefinitefactorfortreatmentandprognosis；TodecideiftheRFi
23、sintheactivephase；Ifarthritisisthemajorpresentations,arthralgiaisntactastheminorpresentations；Ifcarditisisthemajorpresentations，P-Rintervalprolongisntactastheminorpresentations.differentiatediagnosisofRF nJuvenilerheumatoidarthritis(JRA):involvingsmalljoints;Arthritislastingforlongtime;Nomigratory;J
24、ointabnormality.differentiatediagnosisofRFnAcuteleukemia:Fever;Arthralgia;Anemiaorbleedingtendency;Infiltrationsymptoms;Marrowpunctureexamination:diagnosis.differentiatediagnosisofRFnInfectiousendocarditis:Congenitaloracquiredheartdiseasehistory;Anemia;Splenomegaly;Thrombosissymptom;Bloodcultureposi
25、tive;Echocardiography:vegetation.TreatmentofRF nResting:Acutephase:2weeks;CarditiswithoutCHF:23months;CarditiswithCHF:36months.TreatmentofRFnCleaningStreptococcusinfections:Penicillin:2weeks;erythramycin:710d.TreatmentofRFnAntirheumatictreatment:Carditis:Tousecorticosteroidsearlier:Prednisone:1.52mg
26、/kg.d,24w,812w,stop.Methylprednisone:12mg/kg.d,seriouspatients;Beforestopcorticosteroidsapplyaspirintreatmentdosage.TreatmentofRFnAntirheumatictreatment:nocarditis:Aspirin:80100mg/kg.d2w,，Duration:48w.cautionthesideeffects.TreatmentofRFnSymptomatictreatment:congestiveheartfailure：Lowersaltdiet;Inhal
27、ationoxygen;Diuresis:速尿速尿1mg/kg.time;Tonicagents:digoxin;Dilationvessel:regitine;TreatmentofRFnSymptomatictreatment:chorea:Phenobarbital:57mg/kg.dose,Diazepam(安定安定):0.10.3mg/kg.dose.arthralgia:Aspirin：80100mg/kg.d;Toconfinemotivation(制动制动).prophylaxis of RF nPrimaryprevention:Preventionupperairwayin
28、fection;Treatmentstreptococcalinfection.prophylaxis of RFnSecondaryprevention:sufferedrheumaticfeverinthepast：benzathinepenicillin（苄星青霉素）苄星青霉素）120万万u,im,pm510y.rheumaticcarditis:lifetimedrugprophylaxis；Penicillinallergy：macrolide:57dpermonth510y.RF or rheumatic carditis:before and aftersurgerygivean
29、tibiotics1014d.病例分析 10岁患儿，发热近1月，游走性关节痛10余天，伴胸闷，心慌1周。查体：T38，P 150次/分，BP 100/65 mmHg。精神差。扁桃体度肿大。HR 150次/分，心尖部可及2/6级SM。左腕关节、右踝关节肿胀，触痛，活动受限。化验化验：血Rt：WBC16.0109/L，N84%。ESR：90mm/h；EKG：窦性心动过速。病例分析1、请指出下一步应做哪些检查？(ASO,细菌培养)2、请写出诊断及诊断依据。3、请写出鉴别诊断。4、请写出治疗原则。思考题及参考书n初次发生风湿热的诊断标准。n风湿活动性判断。n风湿热的治疗原则和预防措施。v薛新东主编，儿科学，第一版，人民卫生出版社，2002.v杨思源主编，小儿心脏病学，第三版，人民卫生出版社，2005.v胡亚美，江载芳主编，诸福棠实用儿科学，第七版，人民卫生出版社，2002.vBehrman RE主编，Nelson Textbook of Pediatrics,第16版，科学出版社，2001.v汪曾伟，刘维永，张宝仁主编，心脏外科学，人民军医出版社，第一版，2003.