RHEUMATIC FEVER

PRESENTED BY

ADNAN AHMED BHUTTOHamdard University Karachi, Pakistan

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OBJECTIVES

• ETIOLOGY• EPIDEMIOLOGY• PATHOGENESIS• CLINICAL FEATURES• INVESTIGATIONS• DIAGNOSIS• TREATMENT

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ETIOLOGY

• Acute Rheumatic Fever is an inflammatory disease with devastating sequalae.

• It resulting from infection of upper respiratory tract by group A beta hemolytic streptococcal infection.

• It is a diffuse inflammatory disease of connective tissue, primarily involving heart, blood vessels, joints, subcutaneous tissue and brain.

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EPIDEMIOLOGY• Important cause of chronic heart

disease and death in developing countries.

• More common in third world countries.• Commonly occurs in 5-15 years of age.• Male and Female are equally effected.• Overcrowding, poverty, lack of access

to medical care contributes to transmission.

• Incidence is more in cold months

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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, subcutaneous tissue & basal ganglia of brain.

• Antibodies made against group A strep cross react with human tissue.

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Group A Beta Hemolytic Streptococcus

• 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

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Diagrammatic structure of the group A beta hemolytic streptococcus

Cytoplasm

Cyto.membrane

Peptidoglycan

Group carbohydrate

Protein antigens

Cell wall

Capsule

Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain

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

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Rheumatic Carditis Histology

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Histology of Myocardium in Rheumatic Carditis

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CLINICAL FEATURES1. ARTHIRITIS • Most common feature occurs in 80%

of patients.• Flitting & fleeting migratory

polyarthritis, involving major joints• Commonly involved joints knee,

ankle, shoulders, elbow & wrist• Involved joints becomes tender.• In children below 5 yrs arthritis usually mild

but Carditis more prominent• Arthritis do not progress to chronic disease

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04/12/23 12Showing ARTHIRITIS In knee joint

2. Carditis

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• 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)

• Clinical Signs:• High Pulse rate• Murmurs• Cardiomegaly• Rhythm disturbances(Prolonged PR

intervals • Pericardial Friction rubs• Cardiac Failure

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1.Carditis (Contd.)

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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 in Rheumatic heart disease

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3. Sydenham’s Chorea

• Occur in 10-15% of patients with rheumatic fever.

• Mainly in girls resulting from disorder of extra pyramidal system.

• Fast, Clonic, involuntary movements (especially face and limbs)

• Emotionally liability• Muscular Hypotonus• Clinically difficulty in Walking, Talking and

Writing.• It is self limiting but may last from few

weeks to six months and rarely up to 1-2 years.

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•It is self limiting but may last from few weeks to six months and rarely up to 1-2 years.• Chances of reoccurrence are recorded. Shown in table.

3. Sydenham’s Chorea

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4.Subcutaneous Nodules• Occur in 5% of patients • Painless, pea-sized, palpable

nodules.• Mainly over extensor surfaces of

joints, spine, scapulae & scalp.• Associated with strong seropositivity• Always associated with severe

Carditis

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5.Erythema Marginatum

• Occur in 5%of children.• 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

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OTHER CLINICAL FEATURES

• Fever• Arthralgia• Epistaxis• Abdominal pain due to Peritonitis• Hematuria due to involvement of kidneys• Pneumonitis• Mild Pleuritis (5-10%)• Encephalitis (extremely rare)

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LABORTARY INVESTIGATIONS•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

• 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

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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.

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

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Differential diagnosis

• Juvenile rheumatoid arthritis

• Septic arthritis

• Sickle-cell arthropathy

• Kawasaki disease

• Myocarditis

• Scarlet fever

• Leukemia04/12/23 28

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

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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)

Step II: Anti inflammatory treatment

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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

Clinical condition Drugs

Step III: Supportive Management & Management of complications

• Bed rest • Treatment of congestive cardiac

failure: -digitalis, diuretics• Treatment of chorea:

diazepam or haloperidol • Rest to joints & supportive

splinting

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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* intramuscularor

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 Duration

Rheumatic 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

*Clinical or echocardiographic evidence.

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 valvular lesions

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