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RHEUMATIC FEVER
PROF. A K M MOHIBULLAHMBBS; MD (CARDIOLOGY); FACP; FRCP; FACC
DIRECTOR AND PROFESSORNATIONAL INSTITUTE OF CARDIOVASCULAR DISEASES
SHER-E-BANGLA NAGAR, DHAKA
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INTRODUCTION
Acute rheumatic fever is a systemic disease of childhood, often
recurrent that follows group A beta hemolytic (GABH)
streptococcal infection.
It is a delayed non-suppurative sequel to URTI with GABH
streptococci.
It is a diffuse inflammatory disease of connective tissue,
primarily involving heart, blood vessels, joints, subcutaneous
tissue and CNS.
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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, subcutaneoust issue and basal gang l ia of b rain.
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Group A Beta Hemolytic Streptococcus
Strains producing rheumatic fever: M types l, 3, 5, 6, 18 & 24.
Pharyngitis: Produced by GABHS can lead to- acute rheumatic
fever, rheumatic heart disease & post-streptococcalGlomerulonephritis.
Skin infection: Produced by GABHS leads to post streptococcal
glomerulonephritis only. It will not result in Rheumatic Fever orcarditis as skin lipid cholesterol inhibit antigenicity.
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Diagrammatic structure of the group A
beta hemolytic streptococcus
Capsule
Cell wall
Proteinantigens
Group carbohydrate
Peptidoglycan
Cyto. membrane
Cytoplasm
Antigen of outer
protein cell wall
of GABHS
induces antibody
response in
victim whichresult in
autoimmune
damage to heart
valves,
subcutaneous
tissue, tendons,
joints & basal
ganglia of brain
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Pathologic Lesions
Fibrinoid degeneration of connective tissue,
inflammatory edema, inflammatory cell infiltration &
proliferation of specific cells resulting in formation
of Ashcoff nodules, resulting in-
-Pancardi t is in th e heart
-Arth r i t is in the jo ints
-Ashcof f nodulesin the subcu taneous t issue
-Basal gangl iar lesions result ing in c ho rea.
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Histology of Myocardium in Rheumatic Carditis (200X)
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NATURAL HISTORY
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Population S.Infection Manifestation Long Term
Epidemic 3% Recurrence
Carditis(42%) Residual RHD(66%)
Endemic 0.3% Recurrence
No carditis (58%) Residual RHD
(8%)
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CarditisCarditis (71%)
No Carditis (29%)
New Damage(38%)
New Damage(0%)
Residual RHD(60%)
ARF
No Carditis
Carditis (7%)
No Carditis (93%)
New Damage(46%)
New Damage(0%)
Residual RHD(2%)
60% Recurrence
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CLINICAL PRESENTATION
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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 years, arthritis usually mildbut carditis more prominent
Arthritis do not progress to chronic disease.
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Carditis
Manifests as pancarditis (endocarditis, myocarditis andpericarditis), occur in 40-50% of cases.
Cardit is is the on ly manifestat ion o f rheumatic fever that leaves
a sequel & permanent damage to the organ.
Valvul i t is occu r in acute phase.
Chron ic phase- f ibros is, calci f icat ion & stenos is of h eart valves
(f ish-mouth valves).
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Rheumatic
heart
disease.
Abnormal
mitral valve.
Thick, fused
chordae.
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Another view of
thick and fused
mitral valves inRheumatic
heart disease
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Sydenham Chorea
Occurs 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.
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Erythema Marginatum
Occur in
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Subcutaneousnodules
Occur in 10%.
Painless, pea-sized, palpable nodules.
Mainly over extensor surfaces of joints,
spine, scapulae & scalp.
Associated with strong seropositivity.
A lways assoc iated w ith severe cardi t is .
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Other features (Minor features)
Fever (upto 101
degree F)
Arthralgia
Pallor
Anorexia
Loss of weight.
Si lifi d h f h di i f h i di i
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Criteria First attacks Recurrences
Valvulits New onset
Apical systolic murmur
Carey Coombs murmur
Aortic regurgitation murmur
Change in murmur
New onset murmur
Myocarditis Cardiomegaly
CHF/gallop soundsUnexplained tachycardia
Worsening
cardiomegaly
Worsening CHF
Pericarditis Pericardial rubs
Unexplained pericardial
effusion
Pericardial rubs
pericardial effusion
Miscellaneous methods
Conduction disturbancesEchocardiographic imaging
Nuclear imaging
Morphologic evidence at
surgery Histologic evidence at
biopsy or pathology
Simplified schema for the diagnosis of acute rheumatic carditis
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LABORATORY INVESTIGATION
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High ESR
Anemia, leucocytosis
Elevated C-reactive protein
ASO titre >200 Todd units.(Peak value attained at 3 weeks, then
comes down to normal by 6 weeks)
Anti-DNAse B testThroat culture: GABH streptococci.
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ECG: Prolonged PR interval, 2nd or 3rd degree blocks,
ST depression, T inversion.
Echocardiography: Valve edema, mitral regurgitation,
LA & LV dilatation, pericardial effusion, decreased
contractility.
Radionuclide imaging
Endomyocardial biopsy.
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DIAGNOSIS
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Rheumatic fever is mainly a clinical
diagnosis
No single diagnostic sign or specificlaboratory test available for diagnosis
Diagnosis based on MODIFIED JONES
CRITERIA.
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Jones Criteria (Revised) for Guidance in theDiagnosis of Rheumatic Fever*
Major Manifestation Minor Manifestations Supporting Evidenceof Streptococal InfectionClinical LaboratoryCarditis
PolyarthritisChorea
Erythema MarginatumSubcutaneous Nodules
Previousrheumaticfever orrheumaticheart disease
ArthralgiaFever
Acute phasereactants:Erythrocytesedimentationrate,
C-reactiveprotein,leukocytosisProlonged P-
R interval
Increased Titer of Anti-Streptococcal Antibodies ASO
(anti-streptolysin O),
others
Positive Throat Culturefor 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 ofGroup A streptococcal nfection.
Recommendations of the American Heart Association
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Exceptions to Jones Criteria
1. Chorea alone, if other causes have been
excluded
2. Insidious or late-onset carditis with no other
explanation
3. Patients with documented RHD or prior
rheumatic fever, one major criterion, or of
fever, arthralgia or high CRP suggests
recurrence.
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2002-2003 WHO CRITERIA FOR DIAGNOSING
RHEUMATIC FEVER & RHEUMATIC HEART DISEASE
(BASED ON REVISED JONES CRITERIA)
MAJOR MANIFESTATIONS:
Carditis
Polyarthritis
Chorea
Erythema marginatum
Subcutaneous nodule
MINOR MANIFESTATIONS:
Clinical: Fever, polyarthralgiaLaboratory: Elevated acute phase reactants (ESR or
leucocyte count)
ECG: Prolonged PR interval.
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SUPPORTING EVIDENCE OF PRECEDING
STREPTOCOCCAL INFECTION WITHIN THE LAST 45
DAYS:
Elevated or raising ASO or other streptococcal
antibody titre, or
A positive throat culture, or
Rapid antigen test for group A streptococci, or
Recent scarlet fever.
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DIAGNOSTIC CATEGORY CRITERIA
Primary episode of RF 2 major or 1 major and 2 minor
manifestations PLUS evidence of
preceding group A streptococcal
infection
Recurrent attack of RF without
established RHD
2 major or 1 major and 2 minor
manifestations PLUS evidence of
preceding group A streptococcalinfection
Recurrent attack of RF with
established RHD
2 minor manifestations PLUS
evidence of preceding group A
streptococcal infection
Rheumatic chorea
Insidious onset rheumatic carditis
Other major manifestations or
evidence of streptococcal infection
not required
Chronic valve lesions of RHD Do not require any other criteria
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DIFFERENTIAL DIAGNOSIS
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Rheumatiod arthritis
Septic arthritis
Sickle-cell arthropathy
Myocarditis
Leukemia.
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TREATMENT
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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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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 Aspirin75-100mg/kg/day,give as 4divided doses for 6weeks(Attain a blood level 20-
30 mg/dl)Carditis Prednisolone 2-2.5
mg/kg/day, give as twodivided doses for 2weeksTaper over 2 weeks &while tapering add
Aspirin 75 mg/kg/dayfor 2 weeks.Continue aspirin alone100 mg/kg/day foranother 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 haloperidolRest to joints & supportive splinting.
3.Step III: Supportive management &
management of complications
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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
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Duration of Secondary Rheumatic Fever Prophylaxis
Category DurationRheumatic 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
*Cl in ical or echocardiographic evidence.
Recommendations of American Heart Association
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PROGNOSIS
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Rheumatic fever can recur whenever the individual
experience new GABH streptococcal infection, if noton 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.
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