儿科风湿热(RF) 课件

1、 风湿热 (Rheumatic fever)山东大学齐鲁医院儿科李福海 Summarization of rheumatic feverDefinition: 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 in

2、fection;(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 feverIncidence:Related to purulent tonsillitis and scarlet;Applying penicil

3、lin in time 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)Group A -hemolytic streptococcus infection caused isthmitis(咽峡炎),

4、then result in the autoimmune disease.RF发病与A组B型溶链特殊结构成分和细胞外产物有关Pathogenesis of RF molecular mimicry of strep. antigen (链球菌抗原的分子模拟):多种溶链的Ag与发病有关：荚膜透明质酸与人体关节、滑膜有共同Ag关节症状；细胞壁外层M蛋白和M相关蛋白等与人体心肌、心瓣膜糖蛋白有共同Ag心脏症状；细胞膜脂蛋白与人体心肌纤维膜和丘脑下核、尾状核之间有共同Ag中枢神经系统症状；Ag+Ab清除溶链or 器官损伤。Pathogenesis of RFimmunocomplex causes

5、diseases. （免疫复合物致病）Streptococcus Ag + anti-Streptococcus Ab CIC(circulatory immunocomplex) 沉积于 joint synovium (关节滑膜)、myocardium or cardiac valve (+)Complement inflammatory reaction Pathogenesis of RF cellular mediated immune injury(细胞免疫).In vitro reaction of patients peri-lymphocyte to strep Ag was

6、enhanced；Cytotoxic effects of Patients monocyte on cultured myocardium cells;Increased NK cell cytotoxicity ；Abnormal reaction of Tonsil monocyte to strep Ag.Pathogenesis of RF hereditary pathogenesis hereditary susceptibility individual difference of immune responseRF suffers：Indian HLA-B35 America

7、n HLA-DR2 White people HLA-DR4 99% RF patients lymphocyte specific cell alloantigen(异抗原) is positive .（14）Pathology of RF Pathologic phases lasting timeExudative phase(渗出期) 34 weeksHyperplasia phase(增生期) 34 monthsSclerosis phase(硬化期) 23 monthsinvolved organs exudation phase : cardio ; joint synovium

8、; skin;hyperplasia phase: myocardium and cardiac valve(心肌、心瓣膜); pericardial membrane(心外膜); epitenon(腱鞘);Peripheral subcutaneous tissue(关节周围皮下T).Sclerosis phase: mitral valve: stenosis or insufficiencyaortic valve: stenosis or insufficiencyPathologic findingsExudation phase: degeneration; edema; infl

9、ammatory cells infiltration; fibrous exudation; hyperplasia phase: Aschoff small body or rheumatic granuloma Sclerosis phase: Scar development; mitral or aortic valve stenosis or insufficiency Pathologic findingsMyocardial Aschoff body the cells are large, elongated, with large nuclei; some are mult

10、inucleate clinical manifestations of RFSudden onset, carditis and chorea most express chronic course;The degree of clinical manifestations are differently;Latent time: isthmitis, 1to several weeks;Active phase(no treatment): 6m;Recurrent onset (no prophylaxis);clinical manifestations of RF Common ma

11、nifestations:Fever;Fatigue;Pale;epistaxis(鼻出血); abdominal pain; rheumatic pleurisy(风湿性胸膜炎);Pneumonia. clinical manifestations of RFArthritis: 70%75multiplex: large joints: redness, swelling, heat, severe pain. migratory:Lasting ：13w;Arthralgia(关节痛);No joint deformity.clinical manifestations of RF Ca

12、rditis: 40%50% myocarditis: Mild patients have no symptoms;CHF(1015%): HR, heart sound dullness, gallop;cardiac dilation：LV enlarged;cardiac murmur: systolic (mitral) or diastolic (aortic valve), 23/6 degree.EKG: P-R interval, T wave arrhythmia.度AV blockST-T changes of RFArrhythmia of RF clinical ma

13、nifestations of RFEndocarditis: mitral insufficiency :apex 23/6 pansystolic murmur.aortic valve insufficiency: diastolic murmur;mitral relative stenosis: diastolic murmur. Stenotic mitral valve seen from left atrium. Both commissures are fused; the cusps are severely thickened. The left atrium is hu

14、ge. The valve is both incompetent and stenotic 心脏瓣膜病心脏瓣膜病Stenotic mitral valve seen from left atrium, showing fusion of commissures, thickening and calcification of the cusps clinical manifestations of RF Pericarditis: thorax pain;pericardial friction rub;pericardial effusion: chest X-ray or Echocar

15、diogram can diagnosis;pericardial tamponade; EKG: lower voltage, ST segment ;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可见液性暗区 between LV PW and pericardial can see fluid area (effusion)

16、. AVR外所有常规导联中，ST段弓背向下抬高心电图clinical manifestations of RFSydenham chorea: 515 y, female;Extrapyramidal(锥体外系) system signs:Psychologic manifestations; mild patient persist 34 weeks; serious patient persist 34 months；40% accompanied with carditis.clinical manifestations of RFSkin symptoms: 5% erythema m

17、arginatum: 环形、半环形边界清楚粉红色斑；不痛、不痒；一分钱币大小，中心白，边缘高；躯干、四肢近端屈侧；一过性，时隐时现。clinical manifestations of RFErythema marginatum on the trunk, showing erythematous lesions with pale centers and rounded or serpiginous margins clinical manifestations of RFCloser view of erythema marginatum in the same patient clini

18、cal manifestations of RFSkin symptoms:subcutaneous nodules:圆形、硬、无痛结节；粟米至黄豆大小；与皮肤无粘连；见于肘、膝、腕、踝等关节伸面；24周消失。clinical manifestations of RF Subcutaneous nodules are rarely seen and when present, they are usually associated with severe carditis. They are painless, firm, movable, measuring around 0.5 to 2

19、cm. They are usually located over extensor surfaces of the joints, particularly knees, wrists and elbows laboratory examination of RF The proofs of streptococcus infection:throat swab culture: 1/3 streptococcus G +;serum antistreptolysin O test: 80%+;antideoxyribonuclease(anti-DNase) B Ab: 95%+;Anti

20、streptokinase(ASK,抗链球菌激酶) Ab:95%+;anti-hyaluronidase antibody:95%+. （抗透明质酸酶） laboratory examination of RF Rheumatic fever active indicators: WBC ; Neutrophil ; Anemia ; ESR ; CRP (+); 2-globulin . Chorea 以上指标为（）。Diagnosis of RF(Jones 1992) Major manifestations(主要表现): carditis;Polyarthritis;Sydenham

21、chorea;Erythema marginatum;Subcutaneous nodules. Diagnosis of RFMinor manifestations(次要表现):Fever;Arthralgia（关节痛）;Post rheumatic fever history;ESR、CRP+;P-R interval prolonged.Diagnosis of RFProofs of streptococcal infections:Throat swab culture(+);Quickly streptococcal antigen(+);ASO titer ；Recent sc

22、arlet fever history.Definite diagnosis criteria: two major presentations； one major presentation plus two minor presentations； both have the proofs of streptococcus infection.Definite diagnosis:Cautions：Excluding other diseases；Carditis is the definite factor for treatment and prognosis；To decide if

23、 the RF is in the active phase；If arthritis is the major presentations, arthralgia isnt act as the minor presentations；If carditis is the major presentations，P-R interval prolong isnt act as the minor presentations. differentiate diagnosis of RF Juvenile rheumatoid arthritis (JRA): involving small j

24、oints;Arthritis lasting for long time;No migratory;Joint abnormality. differentiate diagnosis of RFAcute leukemia:Fever;Arthralgia;Anemia or bleeding tendency;Infiltration symptoms;Marrow puncture examination:diagnosis. differentiate diagnosis of RFInfectious endocarditis:Congenital or acquired hear

25、t disease history;Anemia;Splenomegaly;Thrombosis symptom;Blood culture positive;Echocardiography: vegetation.Treatment of RF Resting:Acute phase: 2 weeks;Carditis without CHF: 23 months;Carditis with CHF: 36 months.Treatment of RFCleaning Streptococcus infections: Penicillin: 2 weeks; erythramycin:

26、710d. Treatment of RFAntirheumatic treatment: Carditis: To use corticosteroids earlier: Prednisone:1.52mg/kg.d, 24w, 812 w, stop .Methylprednisone:12mg/kg.d,serious patients;Before stop corticosteroids apply aspirin treatment dosage.Treatment of RFAntirheumatic treatment:no carditis: Aspirin:80100mg

27、/kg.d2 w,， Duration: 48w. caution the side effects.Treatment of RF Symptomatic treatment:congestive heart failure：Lower salt diet;Inhalation oxygen;Diuresis:速尿1mg/kg.time;Tonic agents: digoxin ;Dilation vessel: regitine;Treatment of RFSymptomatic treatment:chorea: Phenobarbital: 57mg/kg.dose, Diazepam(安定):0.10.3mg/kg.dose.arthralgia: Aspirin：8010