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