Rheumatic Fever

Mohamed Waheed Elsharief MBBS, MSc MD

Dept of Paediatrics Faculty of Medicine Jazan

objectives

l By the end of this lecture the student should l Define Rheumatic fever and Rheumatic

heart disease (RF ) l Know the epidemiology. Pathogenesis

presentation and diagnosis l Outline the management i.e treatment

prevention l Know the prognosis

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Rheumatic Fever (RF)

Definition: l Rheumatic fever (RF) is an autoimmune

disease affecting the heart and extra- cardiac sites (joints, brain, skin and others)

Epidemiology

l  Ages 5-15 yrs are most susceptible l  Rare <3 yrs l  Girls>boys l  Common in 3rd world countries l  Environmental factors-- over crowding,

poor sanitation, poverty, l  Incidence more during fall ,winter & early

spring 4

Pathogenesis

l Delayed immune response to infection with group.A beta hemolytic streptococci.

l After a latent period of 1-3 weeks, antibody induced immunological damage to heart valves, joints, subcutaneous tissue & basal ganglia of brain

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l Strains that produces rheumatic fever - M types l, 3, 5, 6,18 & 24

l Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis

l Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh.Fever or carditis

Group A Beta Hemolytic Streptococcus

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Clinical Features

l Migratory polyarthritis, involving major joints l Commonly involved joints-knee,ankle,elbow

& wrist l Occur in 80%,

l  In children below 5 yrs arthritis usually mild but carditis more prominent

l Arthritis do not progress to chronic disease

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1.Arthritis

Polyarthritis

Affected Joints are :- Swollen Red

painful Tender to touch Of limited movement

Clinical Features (Contd)

l Manifest as pancarditis(endocarditis, myocarditis and pericarditis),occur in 40-50% of cases

l Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ

l Valvulitis occur in acute phase l Chronic phase- fibrosis,calcification &

stenosis of heart valves. 11

2.Carditis

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Clinical Features (Contd)

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l Occur in 5-10% of cases l Mainly in girls of 1-15 yrs age l May appear even 6 months after the attack

of rheumatic fever l Clinically manifest as-clumsiness,

deterioration of handwriting,emotional lability or grimacing of face

3.Sydenham Chorea

Clinical Features (Contd)

l Occur in <5%. l Unique, transient lesions of 1-2 inches in

size l Pale center with red irregular margin l More on trunks & limbs & non-itchy l Worsens with application of heat l Often associated with chronic carditis

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4.Erythema Marginatum

Clinical Features (Contd)

l Occur in 10% l Painless,pea-sized,palpable nodules l Mainly over extensor surfaces of

joints,spine,scapulae & scalp l Associated with strong seropositivity l Always associated with severe carditis

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5.Subcutaneous nodules

Clinical Features (Contd)

l Fever – Low grade l Arthralgia l Pallor l Anorexia l Loss of weight

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Other features (Minor features)

Laboratory Findings l High ESR l Anemia, leucocytosis l Elevated C-reactive protien l ASO titre >200.

(Peak value attained at 3 weeks,then comes down to normal by 6 weeks)

l  Anti-DNAse B test l  Throat culture-GABHstreptococci

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Laboratory Findings (Contd)

l ECG- prolonged PR interval

l Echo - valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility

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Diagnosis

l Rheumatic fever is mainly a clinical diagnosis

l No single diagnostic sign or specific laboratory test available for diagnosis

l Diagnosis based on MODIFIED JONES CRITERIA

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Jones Criteria (Revised) for Guidance in the Diagnosis of Rheumatic Fever*

Major Manifestation Minor

Manifestations Supporting Evidence of Streptococal Infection

Carditis Polyarthritis

Chorea Erythema

Marginatum Subcutaneous

Nodules

Clinical Laboratory

Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O), others Positive Throat Culture for Group A Streptococcus Recent Scarlet Fever

Previous rheumatic fever or rheumatic heart disease Arthralgia Fever

Acute phase reactants: Erythrocyte sedimentation rate, C-reactive protein, leukocytosis Prolonged P-R interval

*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

Treatment l Step I - primary prevention

(eradication of streptococci) l Step II - anti inflammatory treatment

(aspirin,steroids) l Step III- supportive management &

management of complications l Step IV- secondary prevention

(prevention of recurrent attacks)

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Dr.Said Alavi 23

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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l Bed rest l Treatment of congestive cardiac failure:

-digitalis,diuretics l Treatment of chorea:

-diazepam or haloperidol l Rest 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

Prognosis

l Rheumatic fever can recur not on prophylactic medicines

l Good prognosis for older age group & if no carditis during the initial attack

l Bad prognosis for younger children & those with carditis with valvar lesions

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Summary

l  It is a post GABH strpectoccocal an inflammatory disease which affects the heart joints brain skin diagnosed clinically by two major criteria or one major plus two minor according ducket

l Licks the Joints and Bites the Heart)

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Myocardial Sarcolemma Subthalamic & Caudate Nudeii

Valvular Tissue (glycoprotein)

Myocardium (SACROLEMAL MEMBRAN)

Joint ARTICULAR CARTILAGE

Immune Cross-reactivity

Questions??????

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