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Faculty of Medicine Universitas Brawijaya

Rheumatic Fever. Faculty of Medicine Universitas Brawijaya. Objectives. Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References. Etiology.

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Faculty of Medicine Universitas Brawijaya

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  1. Rheumatic Fever Faculty of Medicine UniversitasBrawijaya

  2. Objectives • Etiology • Epidemiology • Pathogenesis • Pathologic lesions • Clinical manifestations & Laboratory findings • Diagnosis & Differential diagnosis • Treatment & Prevention • Prognosis • References

  3. Etiology • Acute rheumatic fever is a systemic disease of childhood,often recurrent that follows group A beta hemolytic streptococcal infection • It is a delayed non-suppurative sequelae to URTI with GABH streptococci. • It is a diffuse inflammatory disease of connective tissue,primarily involving heart,blood vessels,joints, subcut.tissue and CNS

  4. Epidemiology • Ages 5-15 yrs are most susceptible • Rare <3 yrs • Girls>boys • Common in 3rd world countries • Environmental factors-- over crowding, poor sanitation, poverty, • Incidence more during fall ,winter & early spring

  5. Pathogenesis • Delayed immune response to infection with group.A beta hemolytic streptococci. • After a latent period of 1-3 weeks, antibody induced immunological damage occur toheart valves,joints, subcutaneous tissue & basal ganglia of brain

  6. Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24 Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis as skin lipid cholesterol inhibit antigenicity Group A Beta Hemolytic Streptococcus

  7. Diagrammatic structure of the group A beta hemolytic streptococcus Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain Capsule Cell wall Proteinantigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………………...

  8. Pathologic Lesions • Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules, resulting in- -Pancarditis in the heart -Arthritis in the joints -Ashcoff nodulesin the subcutaneous tissue -Basal gangliar lesions resulting in chorea

  9. Rheumatic Carditis Histology (40X)

  10. Histology of Myocardium in Rheumatic Carditis (200X)

  11. Clinical Features 1.Arthritis • Flitting & fleeting migratory polyarthritis, involving major joints • Commonly involved joints-knee,ankle,elbow & wrist • Occur in 80%,involved joints are exquisitely tender • In children below 5 yrs arthritis usually mild but carditis more prominent • Arthritis do not progress to chronic disease

  12. Clinical Features (Contd) 2.Carditis • Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases • Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ • Valvulitis occur in acute phase • Chronic phase- fibrosis,calcification & stenosis of heart valves(fishmouth valves)

  13. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae

  14. Another view of thick and fused mitral valves in Rheumatic heart disease

  15. Occur in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6/12 after the attack of rheumatic fever Clinically manifest as-clumsiness, deterioration of handwriting,emotional lability or grimacing of face Clinical signs- pronator sign, jack in the box sign , milking sign of hands Clinical Features (Contd) 3.Sydenham Chorea

  16. Clinical Features (Contd) 4.Erythema Marginatum • Occur in <5%. • Unique,transient,serpiginous-looking lesions of 1-2 inches in size • Pale center with red irregular margin • More on trunks & limbs & non-itchy • Worsens with application of heat • Often associated with chronic carditis

  17. Clinical Features (Contd) • Occur in 10% • Painless,pea-sized,palpable nodules • Mainly over extensor surfaces of joints,spine,scapulae & scalp • Associated with strong seropositivity • Always associated with severe carditis 5.Subcutaneous nodules

  18. Clinical Features (Contd) Other features (Minor features) • Fever-(up to 101 degree F) • Arthralgia • Pallor • Anorexia • Loss of weight

  19. Laboratory Findings • High ESR • Anemia, leucocytosis • Elevated C-reactive protien • ASO titre >200 Todd units. (Peak value attained at 3 weeks,then comes down to normal by 6 weeks) • Anti-DNAse B test • Throat culture-GABHstreptococci

  20. Laboratory Findings (Contd) • ECG- prolonged PR interval, 2nd or 3rd degree blocks,ST depression, T inversion • 2D Echo cardiography- valve edema,mitral regurgitation, LA & LV dilatation,pericardialeffusion,decreased contractility

  21. Diagnosis • Rheumatic fever is mainly a clinical diagnosis • No single diagnostic sign or specific laboratory test available for diagnosis • Diagnosis based on MODIFIED JONES CRITERIA

  22. Recommendations of the American Heart Association

  23. Exceptions to Jones Criteria • Chorea alone, if other causes have been excluded • Insidious or late-onset carditis with no other explanation • Patients with documented RHD or prior rheumatic fever,one major criterion,or of fever,arthralgia or high CRP suggests recurrence

  24. Differential Diagnosis • Juvenile rheumatiod arthritis • Septic arthritis • Sickle-cell arthropathy • Kawasaki disease • Myocarditis • Scarlet fever • Leukemia

  25. Treatment • Step I- primary prevention (eradication of streptococci) • Step II- anti inflammatory treatment (aspirin,steroids) • Step III- supportive management & management of complications • Step IV- secondary prevention (prevention of recurrent attacks)

  26. STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Mode Duration Benzathine penicillin G 600 000 U for patients Intramuscular Once 27 kg (60 lb) 1 200 000 U for patients >27 kg or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2-3 times daily For individuals allergic to penicillin Erythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d) or Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d) Recommendations of American Heart Association Dr.Said Alavi

  27. Step II:Anti inflammatory treatment Clinical condition Drugs

  28. Bed rest Treatment of congestive cardiac failure: -digitalis,diuretics Treatment of chorea: -diazepam or haloperidol Rest to joints & supportive splinting 3.Step III: Supportive management & management of complications

  29. STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Mode Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral 1.0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of American Heart Association Dr.Said Alavi

  30. Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence. Recommendations of American Heart Association

  31. Prognosis • Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection,if not on prophylactic medicines • Good prognosis for older age group & if no carditis during the initial attack • Bad prognosis for younger children & those with carditis with valvar lesions

  32. Thank You

  33. VALVULAR HEART DISEASE MITRAL STENOSIS G

  34. ETIOLOGYRHEUMATIC VALVULAR DISEASE • MOST COMMON CAUSE OF M ITRAL STENOSIS • Pure mitral stenosis 25% • Pure mitral regurgitation 35% • Combined MS and MR 40% • 15 TO 20 YEAR LATENCY PERIOD

  35. ETIOLOGYOTHER CAUSES • CONGENITAL • MALIGNANT CARCINOID • SLE OR RHEUMATOID ARTHRITIS • AMYLOID • METHYLSERGIDE THERAPY

  36. PATHOLOGYSYMPTOMATIC MITRAL STENOSIS • THICKENED MITRAL CUSPS • +/- CALCIFIC DEPOSITS • FUSION OF VALVE COMMISSURES • SHORTENING OF CHORDAE WITH FUSION • “FISH MOUTH” OR FUNNEL ORIFICE

  37. HISTORY • PRINCIPLE SYMPTOM IS DYSPNEA • Reduces compliance of the lung • PULMONARY EDEMA • Effort, emotional stress, infection, fever, pregnancy • ATRIAL FIBRILLATION • Increased rate causes increased LA to LV gradient

  38. HISTORY • CHEST PAIN • 15% DUE TO RV HTN, EMBOLIZATION • THROMBOEMBOLISM • 20% HISTORICALLY INVOLVED • CORRELATES INVERSELY WITH CARDIAC OUTPUT • CORRELATES DIRECTLY WITH LA SIZE AND AGE OF PATIENT

  39. PHYSICAL EXAMINATION • ARTERIAL PULSE NORMAL OR DIMINISHED • JUGULAR PRESSURE PROMINENT a WAVE • PALPATION • INCONSPICUOUS LV, RV HEAVE IN PULMONARY HTN

  40. PHYSICAL EXAMINATIONAUSCULTATION • ACCENTUATED S1 • PROLONGED Q-S1 INTERVAL • OPENING SNAP • SUDDEN TENSING OF VALVE LEAFLETS • A2-OS INTERVAL SHORTENS WITH SEVERITY • DIASTOLIC MURMUR

  41. PATHOPHYSIOLOGY • NORMAL VALVE AREA 4 TO 6cm2 • NORMAL MEAN LA TO LV PRESSURE GRADIENT 2 TO 4mmHg • MILD MITRAL STENOSIS 2cm2 • CRITICAL MITRAL STENOSIS 1cm2 or less • 20MMhg GRADIENT REQUIRED FOR FLOW

  42. MANAGEMENTNATURAL HISTORY • 20 TO 25 YEAR ASYMPTOMATIC PERIOD • 5 YEARS FOR PROGRESSION CLASS II-IV • SURVIVAL • CLASS III 62% 5 YR SURVIVAL • CLASS IV 15% 5 YR SURVIVAL • ASYMPTOMATIC CLASS 1 40% WORSENED OR DIED IN 10 YEARS

  43. MANAGEMENTMEDICAL TREATMENT • RHD PCN AND SBE PROPHYLAXIS • SYMPTOMATIC PATIENTS • ORAL DIURETICS AND ACTIVITY RESTRICTION • BETA BLOCKERS AND LOW HEART RATE • DIGOXIN IN AF AND WITH PULM HTN • ANTICOAGULATION FOR LA SIZE >5.5cm, EMBOLISM OR ATRIAL FIBRILLATION

  44. MANAGEMENTSURGICAL TREATMENT • OPERATE FOR SEVERE SYMPTOMS • CLASS III OR GREATER (SYMPTOMS WITH LESS THAN USUAL ACTIVITY) • PULMONARY HTN DEMANDS OPERATION • ROUTINE CATHETERIZATION MEN>45 • MILDY SYMPTOMATIC PATIENTS • CONSIDER SIZE OF MV ORIFICE, LIFESTYLE AND HISTORY OF COMPLICATIONS

  45. MANAGEMENTBALLOON VALVULOPLASTY • PROCEDURE OF CHOICE IN RIGHT PT • TRANSESOPHAGEAL ECHO HELPFUL IN SORTING OUT WHICH PATIENT • ECHO SCALE OF PREDICTORS RELATES TO THICKENING AND CALCIFICATION OF VALVE • RESULTS COMPARABLE TO SURGERY • MORTALITY 2-3%, MORBIDITY 8-12%

  46. VALVULAR HEART DISEASE MITRAL INSUFFICIENCTY

  47. ETIOLOGYACUTE VS CHRONIC • INFLAMMATORY • DEGENERATIVE • INFECTIVE • STRUCTURAL • CONGENITAL

  48. ETIOLOGYDEGENERATIVE • MYXOMATOUS DEGENERATION OF LEAFLETS • MITRAL VALVE PROLAPSE • MOST COMMON CAUSE OF ACUTE MR IN US ADULTS • MARFAN SYNDROME • CALCIFICATION OF MV ANNULUS

  49. ETIOLOGYINFLAMMATORY • RHEUMATIC HEART DISEASE • ACUTE RHEUMATIC FEVER VS CHRONIC • SYSTEMIC LUPUS ERYTHEMATOSUS • SCLERODERMA

  50. ETIOLOGYSTRUCTURAL • RUPTURED CHORDAE TENDINAE • RUPTURE OR DYSFUNCTION OF PAPILLARY MUSCLES • DILATATION OF MITRAL VALVE ANNULUS • PARAVALVULAR PROSTHETIC LEAK

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