rheumatic fever nicvd

7/27/2019 Rheumatic Fever NICVD

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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



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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.



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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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