1. rheumatic fever
by mbbsppt.com

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

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 (antigenic mimicry)

6. Hemolytic Streptococcus
Group A Beta
Hemolytic Streptococcus
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 glomerulonephitis, no heart involvement

7. Pathologic Lesions
Fibrinoid degeneration of connective tissue,inflammatory edema, inflammatory cell infiltration
Ashcoff nodules formation, resulting in-
-Pancarditis in the heart
-Arthritis in the joints
-Ashcoff nodules in the subcut. tissue
-Basal gangliar lesions resulting in chorea

8. POLYARTHRITIS Migratory – flitting and fleeting
Involves large joints sequentially
Polyarthritis- in adults only a single joint may be affected
Lasts 1-5 weeks , Subsides without residual deformity
Occurs in 75% or patients
Dramatic response of arthritis to therapeutic doses of aspirin or NSAIDs

9. Clinical Features (Contd)
Carditis
Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in % 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)

10. Clinical Features (Contd)
Sydenham Chorea
Occur in 5-10% of cases
Mainly in girls of 1-15 yrs age
May appear even 6 t0 12 yr 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

11. Clinical Features (Contd)
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

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

13. Clinical Features (Contd)
Other features (Minor features)
Fever-(upto 101 degree F)
Arthralgia
Pallor
Anorexia
Loss of weight

14. 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-GABH streptococci

15. Laboratory Findings
ECG- prolonged PR interval, 2nd or 3rd degree blocks, ST depression, T inversion
2D Echo cardiography- valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility, diagnosis of IE, Carditis which is not clinically detectable.

16. Diagnosis Rheumatic fever is mainly a clinical diagnosis
Diagnosis based on MODIFIED JONES CRITERIA
Two major criteria OR
One major and two minor criteria+ evidence of recent streptococcal infection

17. Recommendations of the American Heart Association

18. 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 fever, arthralgia or high CRP suggests recurrence

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

20. 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)

21. STEP I: Primary Prevention
Agent Dose Mode Duration
Benzathine penicillin G U Intramuscular Once <27 kg (60 lb) U , >27 kg or
Penicillin V Children: 250 mg 2-3 Oral d (phenoxymethyl penicillin) times daily Adolescents and adults: 500 mg times daily
For individuals allergic to penicillin
Erythromycin: mg/kg/d 2-4 times daily Oral 10 d Estolate (maximum 1 g/d)
Recommendations of American Heart Association

22. Step II: Anti inflammatory treatment
Clinical condition Drugs

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

24. STEP IV : Secondary Prevention of Rheumatic Fever
Agent Dose Mode
Benzathine penicillin G U every 3 weeks Intramuscular or
Penicillin V 250 mg twice daily Oral
Sulfadiazine g once daily for patients 27 kg (60 lb Oral g once daily for patients >27 kg (60 lb)
For individuals allergic to penicillin and sulfadiazine
Erythromycin 250 mg twice daily Oral

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

26. 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

28. RHEUMATIC HEART DISEASE
Results from single or repeated attacks of RF
Rigidity and deformity of valves resulting in stenosis or incompetence or both
Mitral valve alone in 50%
Mitral + Aortic in 25%
Pure aortic uncommon
History of RF obtained in 60%
Not required IE prophylaxis until unless valve replacement surgery is done.

29. Thank You