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  • All right , provide some little tips You should remember the word – MY HEART to help to review the clinical features of KD. M ,h,e ------------------may respectly respectively ------------------------------------- The whole word may remind us the abnormalities of coronary arteries and heard.

7 international students 7 international students Presentation Transcript

  • Rheumatic Fever 风湿热 Cai Yun Department of Pediatrics The Third Affiliated Hospital of Sun Yat-sen University E-mail : wcyf@163.com
  •  Rheumatic fever is an immunological inflammatory disease  follows infection with certain strains of group A streptococci  easily recur without prophylaxis carditis permanent valvular disease migratory polyarthritis chorea subcutaneous nodules erythema marginatum
  • Epidemiology  incidence:22/100000 in China  season:winter or spring  age:5 – 15y
  • Etiology ☆ a nonsuppurative complication of group A streptococcal infection of the upper respiratory tract ☆ occurs 1 - 4 weeks after convalescence of infection ☆ individual propensity ☆ environmental factors latitude altitude humidity nutrition crowding age Colonies and β-hemolysis
  • Pathogenesis  molecular mimicry of bacterial antigens similarity between bacterial and self molecules as recognized by immune cells leading to across-react with target organs in the body  circulating immune complexes (CIC) circulating immune complexes activate the complement system leading to the inflammatory changes  Genetic proneness HLA-B35、HLA-DR4
  • capsule(synovial membranes) Cell wall polysaccharides ( myocardium, endocardium) cell membrane protein ( myocardium 、subthalamic nucleus、caudate nucleus) Cell wall protein(myocardium, endocardium) The antigens of Group A streptococci and molecular mimicry
  • pathology 急性渗出期(acute exudative period) 1 month connective tissue edemas ,effuse, and degenerate, infiltrated with inflammatory cells. 增生期(proliferative period) 3~4 months Aschoff body in myocardium, muscle, endocardium, subcutaneous tissue 硬化期 (sclerotic period) collagen fiber hyperplasia and scar 2~3 months tissue formation mitral >aortic>tricuspid>pulmonary
  • edema and degeneration of collagen and exudation in pericardium pericardial effusion fibrinous pericarditis acute exudative period
  • Aschoff body in endocardium 中心:fibrinoid necrosis of collagen 外周:lymphocytes, plasma cells and Aschoff giant cells Aschoff giant cell large cells with two or more pale nuclei that have prominent nucleoli. proliferative period
  • mitral valve shows stenotic mitral commissures are thickening distorted valve shows fused; cusps are cusps, adherent fusion of severely commissures with commissures, thi thickened. The calcification and ckening and valve is both thrombus deposition, calcification of incompetent and fusion and shortening the cusps. stenotic. of chordae tendinae. sclerotic period
  • Clinical Manifestation Major clinical manifestations: carditis; polyarthritis; chorea; subcutancous nodules; erythema marginatum Ordinary complaints: fever / arthralgia Duration of acute rheumatic fever: ≤6 months
  • rheumatic carditis Incidence: 40~50% One and only permanent damage Endocarditis Myocarditis Pancarditis Pericarditis Congestive heart failure during the initial episode: 5%~10%
  • Myocarditis  Tachycardia disproportionate to the fever  Congestive heart failure  Gallop rhythm  Soft systolic murmur heard at the apex Before treatment  ECG abnormalitis: arrhythmias; prolongation of the P-R interval; atrioventricular block (AVB)  Cardiomegaly on x-ray after treatment
  • Endocarditis • Mitral regurgitation: Apical systolic murmur at the apex • Relative mitral stenosis: Low-pitched mid- diastolic rumble • Aortic regurgitation: Diastolic murmur in the third costa at the left side of the sternum
  • Pericarditis  Precordial pain  A friction rub  Striking increase in heart size on X-ray  Echocardiography: pericardial effusion >50ml  CT scan
  • Rheumatic arthritis  Incidence: 50%~60%  Acute migratory polyarthritis  Larger joints of the extremities are affected: knee、ankle、elbow、wrist  Red, hot, swollen, exquisitely tender and painful if moved  as one joint recovered, another joint may be involved  arthritis lasts less than 1 month without deformity
  • Chorea  Incidence: 3%~10%  Female>male; 8~12 yeas old  Sudden, aimless, irregular movements of the extremities and facial muscles that subside during sleep and exaggerated by emotions: shrug shoulders make eyes bend knees flex wrist
  • Chorea  Emotional instability: nervous  Muscle weakness and ataxia: clumsy, stumble, handwriting or speech disorders normal choreiform movements
  • erythema marginatum The characteristic rashes consist of an evanescent , pink, erythematous maculae, with a clear center and serpiginous outline. The rash is transient, migratory and nonpruritic , which found primarily on the torso and proximal extremities.
  • subcutaneous nodules Subcutaneous nodules are painless small swellings over bony prominences, primarily over the extensor tendons of the hands, feet, elbows, scalp, scapulae, and vertebrae. Nodules tend to occur in crops and may persist for days to months after the onset of acute rheumatic fever.
  • Other clinical features  Variable sweating fever Abdominal  Tiredness angina  paleness  Pneumonia  Nosebleed
  • Laboratory findings  Blood routine test: WBC↑,mild anemia  Acute phase reactants:ESR↑, CRP↑  Isolation of group A streptococci (+)  Serum antibody against the specific strptococci: ASO↑, ASK ↑, AH ↑, anti-DNase B ↑  Immune system: IgG ↑, IgA ↑, C3 ↑  ECG: P-R interval ↑, second degree AVB  Routine roentgenogram  Echocardiography
  • The Jones Criteria Revised with Addition of World Health Organization Recommendations Major Criteria Minor Criteria Carditis Fever Polyarthritis, migratory Arthralgia Erythema marginatum increased acute-phase reactants Chorea ESR↑, CRP↑ Subcutaneous nodules Prolonged P-R interval Plus Evidence of a preceding group A streptococcal infection (culture, rapid antigen, antibody titers rise/elevation, scarlet fever) ★ two major manifestations + Evidence of S.I (streptococcal infection ) ★ one major+two minor manifestations + Evidence of S.I
  • Dignosis of active rheumatic fever Fever, body weight ↓, tireness Tachycardia or arrhythmias ESR↑, CRP↑, neutrocyte↑, anti body titer↑
  • Differential diagnosis  Fever  Carditis  Arthritis
  • Differential diagnosis of carditis Infective endocarditis: anemia, splenomegaly, petechia, embolism blood culture(+) vegetations on endocardium / valves Viral myocarditis: arrhythmias ( premature contraction) evidence of viral infection
  • Differential diagnosis of arthritis  Systemic lupus erythematosus (SLE) : malar rash, proteinuria, hypertension, leukopenia, Coombs(+) hemolytic anemia, antinuclear antibodies(+)  Juvenile rheumatoid arthritis (JRA): morning stiffness, iridocyclitis, progression of joint destruction, ANA(+), rheumatoid factor(+)
  • Management  Bed rest  antibiotics  anti-rheumatism therapy  heteropathy
  • (1)Bed rest congestive carditis cardiamegaly heart failure -- -- -- 2w 2w + -- -- 4w 4w + + -- 6w 6w + + + 8w 3 mon
  • (2)antibiotics  Procaine penicillin G: 4.8 millon U ~ 9.6 millon U / d,iv drip ×2~3 w  PG AST (+) : Erythromycin p.o ×10 d (3)anti-rheumatism therapy  Carditis:Prednisone, 2mg/kg.d ( ≤ 60mg/d ) ×2~4w; reduce dose gradually; full duration = 8~12 w  arthritis:Aspirin , 80~100mg/kg.d ( ≤ 3g /d ) until remission; gradually reduce to half dose for 4~6w
  • (4) heteropathy congestive heart failure: steroid; oxygen therapy; diuresis; captopril;digitalis ( small dose)  chorea:tranquilizer (chlorpromazine, barbital )  arthralgia: immobilization of affected joints
  • prophylaxis  Recurrent rheumatic fever benzathine Penicillin: 1.2million U , Q4W,≥5 years patients with established heart disease may continue for ≥ 10 years, even the whole life. PG AST (+) : Erythromycin p.o ×6 ~7 d, every month  Bacterial myocarditis Patients with rheumatic heart disease should receive antibiotic prophylaxis before and after operation to prevent bacterial infection.
  • Emphases  Five major clinical manifestations  Jones criteria  Features of active rheumatic fever  treatment:  prophylaxis:long-acting PG
  • Kawasaki disease 川崎病 (Mucocutaneous lymph node syndrome)
  •  Tomisaku Kawasaki described KD in 1967  KD is an acute generalized systemic vasculitis of unknown etiology with fever and rashes.  Coronary artery dilation or aneurysms  KD has replaced acute rheumatic fever as the most common cause of acquired heart disease in children In developed countries
  • Epidemiology  Age:< 4 years old (80%) < 2 years old (50%)  Sex:more often in males than in females (1.5:1)  Season:clusters in winter / spring  Racial background: Asian children, especially those of Japanese descent.
  • Etiology and Pathogenesis  etiology of KD remains undiscovered.  immunopathogenic mechanism for coronary disease  organism super-antigen  mimic antigen ( HSP65)  T cell - mediated immune response  cytokine – mediated immune damage
  • Pathophysiology — systemic vasculitis (coronary arteries)  stageⅠ:1~10 d,acute small periarteritis; cardiac inflammatory changes  stageⅡ:10~25 d,coronary arteritis; elastic laminae and muscular layers split, leading to thrombus and aneurysms.  stage Ⅲ:26~31 d, acute inflammation remission; fibrous tissue proliferates; intima thickens; coronary arteries narrow or occlude .  stage Ⅳ:≧40 d, cicatrization in myocardium; occluded arteries reopen.
  • normal coronary artery stageⅠ stage Ⅱ
  • 10 days after the onset of symptoms, elastic laminae splits , intima proliferates and thickens in branch of coronary artery.
  • Huge coronary artery aneurysm
  • Clinical manifestation  Mucocutaneous lymph node abnormalities  Cardiovascular abnormalities  Other nonspecifically manifestations
  • Main clinical features 1. Fever  usually more than 39°C, for at least 5 days  High spiking and remittent  not responds to antibiotics  Generally persists 1-2 weeks without treatment  usually resolves in 1-2 days after treatment with intravenous gamma globulin (IVIG)
  • Main clinical features 2. Bilateral conjunctival injection without exudate
  • Main clinical features 3. inflammation of the lips and oral cavity  Injected, dry, fissured- lips  injected oral and pharyngeal mucosa  Strawberry tongue with prominent papillae and erythema  no oral exudates, ulcerations, or Koplik spots
  • Main clinical features 4. Hands and feet  Erythema, or indurative edema of palms and soles  Periungual membranous desquamation of fingers and toes about 2 weeks after onset  Transverse grooves across the nails
  • Main clinical features 5. rash of various forms  diffuse, scarlatiniform or erythema polymorphous rash  erythema or desquamation in perineal region
  • Main clinical features 6. non- purulent cervical lymphadenopathy  50-75% of patients  With a node size of 1.5 cm or greater in diameter  tenderness, not red
  • Cardiovascular abnormalities 1. carditis Tachycardia Gallop rhythm systolic murmurs Arrhythmia 2. myocardial ischemia angina myocardial infarction
  • 3. Coronary arterial changes — 2~4 weeks after onset / convalescent phase  coronary arteritis vessel intima roughened  coronary arteries narrow  coronary arteries dilation(CAD)  coronary artery aneurysm
  • Coronary Artery Aneurysm — 20~30% of untreated children LAD Aneurysm at left normal coronary artery anterior descending ( LAD) coronary artery
  • High risk factors of CA aneurysm  age: < 6 month or > 3 years  male sex  fever for more than 16 days or recurrence  cardiomegaly or arrhythmia  lab findings: Hb < 80g/L, WBC > 16~30X109/L, PLT > 1000X109/L, ESR > 100mm/h  KD recurrence
  • Less-common features  aseptic  gallbladder meningitis hydrops  abdominal  hepatic pain dysfunction  otitis media  arthralgia  jaundice  arthritis  diarrhea  urethritis
  • Laboratory findings Blood analysis : WBC↑; mild anemia; PLT↑in 2nd~3th week; ESR↑; CRP↑; ALT↑; AST↑
  • Immune system IgG、 IgM、IgA、IgE↑; Circulating Immune Complexes ↑ ; C3 normal or ↑
  • ECG:ST-T abnormalities of pericarditis or myocardial infarction Chest roentgenogram:nonspecific perihilar or parenchyma infiltrates; cardiamegaly.
  • Echocardiography  coronary arteritis intima roughened  coronary arteries narrow or dilation  coronary artery aneurysm  pericardial effusion  mitral , aortic, or 1. coronary artery aneurysm 2. right coronary artery trunk tricuspid disturbed 3. aorta flow
  •  diameter of normal CA ( main stem) 0 ~ 3 y <2.5 mm 3 ~ 9 y <3 mm 9 ~ 14 y <3.5 mm  grades of CAD ( diameter of CA) mild 3mm ~ 4mm moderate 4mm ~ 7mm severe ≥8mm (CA aneurysm)
  • Coronary angiography — myocardial ischemia / multiple coronary aneurysms normal aneurysm LAD dilation and narrow
  • Diagnostic guidelines (for typical cases) fever lasting more than 5 days + 4 of the following 5 criteria (other illnesses must be excluded): 1. polymorphous rash 2. bilateral conjunctival injection without exudate 3. diffuse injection of oral mucosa, erythema or fissuring of the lips, strawberry tongue 4. nonpurulent cervical lymph node enlargement (one lymph node >1.5 cm) 5. extremity changes: erythema of palms / soles, indurative edema of hands / feet, Membranous desquamation of the fingertips
  • Diagnostic guidelines (for atypical cases)  fever lasting more than 5 days  ≤3 of the 5 criteria  coronary arteries dilation or aneurysm detected by echocardiography
  • Differential diagnosis Scarlet fever Red rash blanches with pressure, which is diffuse but spares the palms, soles, and face. The face appears flushed. The skin rash fades in a week and is followed by extensive desquamation. Patient has good response to PG.
  • Differential diagnosis Exudative and Erythema Multiforme polymorphous Erythema, herpes and extensive desquamation; oral ulcers; conjunctival exudate; no indurative edema of palms or soles
  • Differential diagnosis  septicemia blood curture (+), good response to antibiotics detected focus  systemic onset of juvenile rheumatoid arthritis NO conjunctival injection NO injection of oral mucosa or fissuring of the lips NO Indurative edema of hands and feet , or desquamation NO coronary artery damage
  • Treatment & Medication • relieve vasculitis • inhibit PLT aggregation
  • (1)aspirin  administered for anti-inflammatory and antithrombotic effects  acute phase : 80-100 mg/kg/d PO in tid/qid  72h after defervescence: reduce dose gradually  2 weeks after defervescence: 3-5 mg/kg/d p.o×6~8 weeks until ESR , PLT and coronary arteries return to normal (Ø<3mm)
  • (2) intravenous gammaglobulin ( IVIG)  reduce the prevalence of coronary abnormalities and lead to rapid defervescence  2 g/kg IV infusion over 8-12 h within 10d after onset  Patient with incomplete response can receive a second course of IVIG.  defer using live viral vaccines until about 11 months after IVIG administration
  • (3) corticosteriods  indications of administration pancarditis no available IVIG no response to IVIG  prednisone / methylprednisolone  combination with aspirin + persantine
  • (4) inhibit PLT aggregation  persantine 3~5mg/kg/d  maintenance treatment in patient with huge or multiple coronary aneurysms aspirin 3~5mg/kg/d + warfarin (5) Other therapy  Liquid therapy  thrombolytic medications  coronary artery bypass graft
  • Prognosis  principal cause of death: myocardial infarction  fatality rate:0.5-1%  recurrence rate: 1-2%  incidence of CA aneurysms: 20~30% in untreated patient 15% in IVIG treated patient
  • outpatient follow-up  systemic examination  no CA abnormality: the first 1,3,6,12 month  CA abnormality: the first 1,3,6 month, then every 6-12 month until CA return to normal
  • Emphases  acute generalized systemic vasculitis  coronary artery abnormalities  diagnostic guidelines  treatment: goals / medication  out patient follow-up
  • M(y) Heart  M : mucocutaneous  H : hand / feet  E (eye) :conjunctiva  A : adenopathy (lymphadenopathy)  R : rash  T(tempreture):fever