1. Acute rheumatic fever is a systemic disease that primarily affects the heart, joints, skin, and brain in children following a streptococcal throat infection. 2. It is caused by an autoimmune response 2-3 weeks after streptococcal pharyngitis. 3. Diagnosis is based on the modified Jones criteria and treatment involves antibiotics for streptococcal infections, anti-inflammatory drugs like aspirin and steroids, and long-term antibiotic prophylaxis to prevent recurrent attacks.

1. DR. G. SWARNA LATHA DEVI
M.D
PRO.OF MEDICINE

2. 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
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3. 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 RAINfall ,winter &
early spring
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4. 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 to
heart valves,joints, subcutaneous tissue
& basal ganglia of brain
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 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.
Glomerulonephritis
 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

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Diagrammatic structure of the group A
beta hemolytic streptococcus
Capsule
Cell wall
Protein antigens
Group carbohydrate
Peptidoglycan
Cyto.membrane
Cytoplasm
……………………………………………
……...
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

7. 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 nodules in the subcutaneous
tissue
-Basal gangliar lesions resulting in CHOREA
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8. ACUTE RHEUMATIC FEVER
Redness & swelling
of throat & tonsils;
Beefy, swollen, red
uvula; Soft palate
petechiae
(“doughnut
lesions”)
Tonsillopharyngeal
erythema &
exudates
Sore throat: fever,
white draining
patches on the
throat & swollen or
tender lymph glands
in the neck

9. Histology of Myocardium in Rheumatic
Carditis (200X)
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11. Clinical Features - ACCNE
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
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12. Clinical Features (Contd)
 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)
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2.Carditis

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

14. Clinical Features (Contd)
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 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 hand writing,emotional
instability , grimacing movements of face .
 Clinical signs- pronator sign, jack in the box
sign , milking sign of hands
3.Sydenham Chorea

18. Clinical Features (Contd)
 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
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4.Erythema Marginatum

20. ERYTHEMA MARGINATUM

21. 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
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5.Subcutaneous nodules

22. SUBCUTANEOUS NODULES

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Jones Criteria (Revised) for Guidance in the
Diagnosis of Rheumatic Fever*
Major Manifestation Minor
Manifestations
Supporting Evidence
of Streptococal Infection
Clinical Laboratory
Carditis
Polyarthritis
Chorea
Erythema Marginatum
Subcutaneous Nodules
Previous
rheumatic
fever or
rheumatic
heart disease
Arthralgia
Fever
Acute phase
reactants:
Erythrocyte
sedimentation
rate,
C-reactive
protein,
leukocytosis
Prolonged P-
R interval
Increased Titer of Anti-
Streptococcal Antibodies ASO
(anti-streptolysin O),
others
Positive Throat Culture
for Group A Streptococcus
Recent Scarlet Fever
*The presence of two major criteria, or of one major and two minor criteria,
indicates a high probability of acute rheumatic fever, if supported by evidence of
Group A streptococcal nfection.
Recommendations of the American Heart Association

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

27. 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
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28. 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,pericardial
effusion,decreased contractility
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29. primarily prolonged PR interval

30. Cardiomegaly

31. 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
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32. Differential Diagnosis
 Juvenile rheumatiod arthritis
 Septic arthritis
 Sickle-cell arthropathy
 Myocarditis
 Leukemia
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33. 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)
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34. Dr.Said Alavi 34
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

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Arthritis only Aspirin 75-100
mg/kg/day,give as 4
divided doses for 6
weeks
(Attain a blood level 20-
30 mg/dl)
Carditis Prednisolone 2-2.5
mg/kg/day, give as two
divided doses for 2
weeks
Taper over 2 weeks &
while tapering add
Aspirin 75 mg/kg/day
for 2 weeks.
Continue aspirin alone
100 mg/kg/day for
another 4 weeks
Step II: Anti inflammatory treatment
Clinical condition Drugs

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 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

37. Dr.Said Alavi 37
STEP IV : Secondary Prevention of Rheumatic Fever
(Prevention of Recurrent Attacks)
Agent Dose Mode
Benzathine penicillin G 1 200 000 U every 3 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

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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

39. 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
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