Rheumatic feve

Dr.Said Alavi

MD,DCH,DNB,FCPS

Dr.Said Alavi

MD,DCH,DNB,FCPS

Dept. of Pediatrics and NeonatologyDept. of Pediatrics and Neonatology

Saqr Hospital,Ras Al KhaimahSaqr Hospital,Ras Al Khaimah

UNITED ARAB EMIRATESUNITED ARAB EMIRATES

E-mail: [email protected]: [email protected]

05/05/1999 Dr.Said Alavi2

ObjectivesObjectivesObjectivesObjectives Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory

findings Diagnosis & Differential diagnosis Treatment & Prevention Prognosis References


EtiologyEtiology 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


EpidemiologyEpidemiology

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


PathogenesisPathogenesis

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


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


Diagrammatic structure of the group A beta hemolytic streptococcus

Capsule

Cell wall

Protein antigens

Group carbohydrate

Peptidoglycan

Cyto.membrane

Cytoplasm

…………………………………………………...

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 LesionsPathologic 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


Rheumatic Carditis Histology (40X)


Histology of Myocardium in Rheumatic Carditis (200X)


Clinical FeaturesClinical 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)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


Another view of thick and fused mitral valves in Rheumatic heart disease



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



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



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



Other features (Minor features)

Fever-(upto 101 degree F) Arthralgia Pallor Anorexia Loss of weight


Laboratory FindingsLaboratory 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)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


DiagnosisDiagnosis Rheumatic fever is mainly a clinical

diagnosis No single diagnostic sign or specific

laboratory test available for diagnosis Diagnosis based on MODIFIED

JONES CRITERIA


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

Major Manifestation MinorManifestations

Supporting Evidence of Streptococal Infection

Clinical LaboratoryCarditisPolyarthritis

ChoreaErythema Marginatum

Subcutaneous Nodules

Previousrheumaticfever orrheumaticheart diseaseArthralgiaFever

Acute phasereactants:Erythrocytesedimentationrate, C-reactiveprotein,leukocytosis Prolonged P-R interval

Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O),othersPositive Throat Culture for Group A StreptococcusRecent 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


Exceptions to Jones CriteriaExceptions 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 DiagnosisDifferential Diagnosis

Juvenile rheumatiod arthritis Septic arthritis Sickle-cell arthropathy Kawasaki disease Myocarditis Scarlet fever Leukemia


TreatmentTreatment 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


Arthritis only Aspirin 75-100mg/kg/day,give as 4divided doses for 6weeks(Attain a blood level 20-30 mg/dl)

Carditis Prednisolone 2-2.5mg/kg/day, give as twodivided doses for 2weeksTaper over 2 weeks &while tapering addAspirin 75 mg/kg/dayfor 2 weeks.Continue aspirin alone100 mg/kg/day foranother 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


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

orPenicillin V 250 mg twice daily Oral

orSulfadiazine 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


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.

Recommendations of American Heart Association


PrognosisPrognosis

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


ReferencesReferencesHoffman JIE: Rheumatic Fever . Rudolph's Pediatrics; 20th Ed: 1518 - 1521,1996.

Stollerman GH: Rheumatic Fever . Harrison's Principles Of Internal Medicine; 13th Ed: 1046 - 1052,1995.

Special Writing Group of the Committee on Rheumatic Fever,endocarditis & Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association: Guidelines for the Diagnosis of Rheumatic Fever. In Jones Criteria, 1992 Update JAMA 268:2029,1992

Todd J: Rheumatic Fever . Nelson's Textbook Of Pediatrics; 15th Ed: 754 - 760, 1996.

Warren R, Perez M, Wilking A: Pediatric Rheumatic Diseases . Pediatric Clinics of North America; 41: 783 - 818,1994.

World Health Organization Study Group: Rheumatic Fever & Rheumatic Heart Disease,technical Report Series No. 764.Geneva,world Health Organization, 1988


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Rheumatic feve