Rheumatic Fever
Dr.B.BALAGOBI
Objectives• Introduction• Etiology• Epidemiology• Pathogenesis• Pathologic lesions• Clinical manifestations & Laboratory
findings• Diagnosis & Differential diagnosis• Treatment & Prevention• Prognosis
Acute Rheumatic Fever...• A connective tissue disease• Acquired heart disease• Mainly in Developing countries • Significant morbidity and mortality• Association with pharyngitis - group A
haemolytic streptococci• High risk of recurrence –So prophylaxis is needed
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
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
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
Group A streptococcal pharyngitis
• 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
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
Clinical Features
• 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
1.Arthritis
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)
2.Carditis
Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae
Clinical Features (Contd)
• 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
3.Sydenham Chorea
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
4.Erythema Marginatum
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
Clinical Features (Contd)
Other features (Minor features)
• Fever-(upto 101 degree F)• Arthralgia• Pallor• Anorexia• Loss of weight
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
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
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
Guidelines for diagnosis of the initial attack of rheumatic fever. Duckett Jones criteria, 1992
update - American Heart Association
• 2 major manifestations
or• 1 major and 2 minor manifestations • supported by
– Evidence of antecedent streptococcal infection
Major manifestations...
• Polyarthritis• Carditis• Chorea• Subcutaneous nodules• Erythema marginatum
Minor manifestations...
• Clinical – Arthralgia– Fever
• Laboratory – Elevated acute-phasereactants (ESR,CRP)– Prolonged PR interval
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
Differential Diagnosis
• Juvenile rheumatiod arthritis• SLE• Septic arthritis• Sickle-cell arthropathy• Kawasaki disease• Myocarditis• Scarlet fever• Leukemia
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Management...Average course of 6-8 weeks
• Admit - confirmation, education, drugs• Investigations • Bed rest - CCF - strict bed rest• Antibiotics - oral penicillin for 10 days or IM
Benzathine penicillin • Anti rheumatic drugs - aspirin / steroids• Aspirin - dose/administration/side effects • Duration: RF: ~ 6 weeks and tail off over ~ 2wks
RC: 8 -10 weeks and tail off over ~ 2 wks• Steroids - no effect on long term prognosis
CCF / impending heart failure
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)
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 treatmentClinical condition Drugs
• 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
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Why prophylaxis..?• To prevent streptococcal infections which
precipitate recurrences of rheumatic fever• Prevent development of chronic rheumatic heart
disease• If recurrences are prevented, 70% of patients with
carditis in the initial attack will eventually have normal hearts
• No documented evidence of resistance of group A streptococci to penicillin
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Prophylaxis...
• Primary - · Adequate treatment of streptococcal sore
throats - oral penicillin for 10 days · Clinical differentiation of viral/bacterial
sore throats is difficult · Throat swab for culture and ABST· Erythromycin
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Prophylaxis ctd...• Secondary - • Benzathine penicillin 1.2 mega units IM ( ARF - 4
weekly/RC - 3 weekly )• Duration - ARF - 18 / 21yrs or 5yrs after last attack• Carditis - (extent of damage) ~ 25 • Chronic valvular heart disease - life long• Infective endocarditis prophylaxis - life long
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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T/F In Rheumatic fever?
A. is causing deformity in jointsB. small joints of the hands are commonly
affectedC. Anti streptolysin O is elevatedD. Aspirin treatment prevents the cardiac
involvementE. Sleeping pulse rate is elevated in Carditis
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T/F Features of rhematic carditis?
A. Pericardial rubB. Congestive heart failureC. Coronary artery aneurysmD. Mid diastolic murmurE. tachycardia
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T/F which of the following are the minor criteria of Rheumatic fever?
A. sub cutaneous noduleB. ArthritisC. Elevated ASOTD. Raised ESRE. FeverF. Prolonged PR interval in ECG
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T/F regarding Rheumatic fever?
A. Chorea is associated with subcutaneous nodule
B. Prolong PR interval in ECG indicates the underlying carditis
C. Erythema nodosum is a major criteriaD. IM Benzathine penicillin given 3 weekly if
carditis is presentE. Mitral stenosis is common at the acute stage
of the disease
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T/F regarding Rheumatic fever?
A. Steroids are superior to salicylates in prevention of carditis
B. Subcutaneous nodules are associated with bad prognosis
C. History of sore throat is essential for the diagnosis
D. Can Cause early diastolic murmur at left lower sternal edge
E. Can cause cardiomegaly
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T/F regarding Rheumatic fever?
A. In patient with Rheumatic valvular heart disease antibiotic prophylaxis monthly given to up to 21 years of age
B. In patient with Rheumatic valvular heart disease antibiotic prophylaxis monthly given to prevent infective endocarditis
C. Emotional lability is a feature of ChoreaD. Aortic valve involvement is commoner than mitral
valve involvement.E. New onset Pansystolic murmur is a feature.
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T/F Rheumatic fever?
A. Low dose aspirin is used in the treatmentB. Common in children than adultsC. Cause erosive arthritisD. Seen in 15% of children with phayrngitisE. There are no recurrence
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T/F Rheumatic fever?
A. Associated with β haemolytic streptococciB. Can not be diagnosed if normal ASOTC. Chorea is a late featureD. Commonly affects the endocardium of the
heartE. Chorea is common in boys
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T/F regarding Acute Rheumatic fever?
• Salicylates or steroids should not be started until diagnosis is confirmed
• Antibiotic therapy during acute infection can alter the severity of cardiac involvement
• Compared to salicylates ;steroids use significantly reduce rheumatic valvular disease
• Prophylaxis with Oral penicillin /IM benzathine penicillin are equally effective
• Effective serum concentration of drug detected up to 4 wks after IM Benzathine penicillin