Acute Rheumatic Fever

Acute Rheumatic Fever Presentation, Causes, Treatment, Prognosis

1. Rheumatic fever ( French physician Ernst-Charles Lasègue 1884) “It licks the joints, but bites the heart”. Dr Zain Ul Abidin Bahawal Victoria Hospital Bahawalpur x [email protected]

2. Scheme of Presentation. Aetiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis

3. Aetiology . Acute rheumatic fever is a systemic disease of childhood, often recurrent, that follows infection by group A beta hemolytic streptococci( GABH ).

4. It is a diffuse inflammatory disease of connective tissue , primarily involving heart, blood vessels, joints, subcutaneous tissue and CNS. Epidemiology. Ages 5-15 yrs are most susceptible. Rare under 3 years. 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 immunologically-mediated inflammatory response occurs.

6. Pharyngitis produced by GABHS can lead to acute rheumatic fever, rheumatic heart disease & post-streptococcal Glomerulonephritis .

7. Pathologic Lesions Fibrinoid degeneration of connective tissue, inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in : Pancarditis in the heart with formation of Ashcoff nodules which are  granulomatous structures consisting of  fibrinoid change, lymphocytic infiltration, plasma cells, and characteristically abnormal macrophages surrounding necrotic centres . Arthritis in the joints Nodules in the subcutaneous tissue Basal ganglia lesions resulting in chorea

8. Recommendations of American Heart Association

9. 1. Arthritis Flitting & fleeting migratory polyarthritis , involving major joints Commonly involved joints: knee, ankle, elbow & wrist. Occur in 75%,involved joints are exquisitely tender. In children below 5 yrs arthritis is usually mild but carditis is more prominent. Arthritis does not progress to chronic disease. 2. Carditis Manifests as pancarditis ( endocarditis , myocarditis and pericarditis ),occurs in 40-50% of cases (90% in age 3 yrs & 30% in adolescents) Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ. Valvulitis occurs in acute phase Chronic phase: fibrosis, calcification & stenosis of heart valves ( fishmouth valves)

10. CARDITIS (continued) SIGN & SYMPTOMS : Breathlessness, palpitation and tachycardia, chest pain, cardiac enlargement, new and changed cardiac murmurs. Soft systolic murmur due to MITRAL regurgitation is very common. Soft mid-diastolic murmur (called the CAREY COOMB’S MURMUR, due to valvulitis with nodule formation on the valve leaflets, is characteristic). Aortic valve incompetence is present in 50% of cases. Pulmonary and tricuspid valves are rarely involved.

11. Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae Diseased, incompetent mitral valve with vegetations on the cusps Aschoff bodies are microscopic structures seen in patients with rheumatic fever

12. 3.Sydenham’s Chorea Occurs in 5-10% of cases Mainly in girls of 1-15 yrs age May appear even 6 months after the attack of ARF . Clinically manifests as: clumsiness, deterioration of handwriting, emotional lability or grimacing of face Clinical signs: pronator sign, milking sign of hands.

13. 4. Erythema Marginatum Occurs 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

14. 5. Subcutaneous nodules 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 6. Other features (Minor features) Fever: ( upto 101 degree F) Arthralgia Pallor Anorexia Loss of weight

15. 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 X-RAY CHEST: cardiomegaly and chest congestion ECG : prolonged PR interval, 2nd or 3rd degree blocks, ST depression,T inversion Echo- cardiography : valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility

16. The blood-agar culture plates show a positive streptococcus infection with the bacteria arranged in chains and the halo effect caused by haemolysis shows specifically a beta-hemolytic group A bacteria. Laboratory diagnosis of rheumatic fever ( contd /-)

17. 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.

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

19. 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)

20. STEP I : Primary Prevention of Rheumatic Fever. ( Treatment of Streptococcal Tonsillo-pharyngitis ) 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 estolate 20-40 mg/kg/d 2-4 times daily Oral 10 (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

21. Step II: Anti inflammatory treatment Clinical condition Drugs

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

23. 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

24. 26 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 valvular 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 valvular disease * ) Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic evidence. Recommendations of American Heart Association

25. Prognosis Rheumatic fever can recur whenever the individual experiences 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 valvular lesions

26. To summarise : salient features of ARF :

27. Thank You