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Rheumatic Fever. Dr.Said Alavi MD,DCH,DNB,FCPS. Dept. of Pediatrics and Neonatology Saqr Hospital,Ras Al Khaimah UNITED ARAB EMIRATES E-mail: drsaid@emirates.net.ae. Objectives. Etiology Epidemiology Pathogenesis Pathologic lesions Clinical manifestations & Laboratory findings

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dr said alavi md dch dnb fcps

Rheumatic Fever


Dept. of Pediatrics and Neonatology

Saqr Hospital,Ras Al Khaimah


E-mail: drsaid@emirates.net.ae

  • Etiology
  • Epidemiology
  • Pathogenesis
  • Pathologic lesions
  • Clinical manifestations & Laboratory findings
  • Diagnosis & Differential diagnosis
  • Treatment & Prevention
  • Prognosis
  • References

Dr.Said Alavi

  • 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

Dr.Said Alavi

  • 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

Dr.Said Alavi

  • Delayed immune response to infection with group.A beta hemolytic streptococci.
  • After a latent period of 1-3 weeks, antibody induced immunological damage occur toheart valves,joints, subcutaneous tissue & basal ganglia of brain

Dr.Said Alavi

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

Dr.Said Alavi


Diagrammatic structure of the group A beta hemolytic streptococcus

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


Cell wall


Group carbohydrate





Dr.Said Alavi

pathologic lesions
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 nodulesin the subcutaneous tissue

-Basal gangliar lesions resulting in chorea

Dr.Said Alavi

clinical features
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

Dr.Said Alavi

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)

Dr.Said Alavi

clinical features contd15
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

Clinical Features (Contd)

3.Sydenham Chorea

Dr.Said Alavi

clinical features contd16
Clinical Features (Contd)

4.Erythema Marginatum

  • 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

Dr.Said Alavi

clinical features contd17
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

Dr.Said Alavi

clinical features contd18
Clinical Features (Contd)

Other features (Minor features)

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

Dr.Said Alavi

laboratory findings
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

Dr.Said Alavi

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

Dr.Said Alavi

  • Rheumatic fever is mainly a clinical diagnosis
  • No single diagnostic sign or specific laboratory test available for diagnosis
  • Diagnosis based on MODIFIED JONES CRITERIA

Dr.Said Alavi

exceptions to jones criteria
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

Dr.Said Alavi

differential diagnosis
Differential Diagnosis
  • Juvenile rheumatiod arthritis
  • Septic arthritis
  • Sickle-cell arthropathy
  • Kawasaki disease
  • Myocarditis
  • Scarlet fever
  • Leukemia

Dr.Said Alavi

  • 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)

Dr.Said Alavi


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)


Ethylsuccinate 40 mg/kg/d 2-4 times daily Oral 10 d (maximum 1 g/d)

Recommendations of American Heart Association

Dr.Said Alavi


Step II:Anti inflammatory treatment

Clinical condition Drugs

Dr.Said Alavi

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

Dr.Said Alavi


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


Penicillin V 250 mg twice daily Oral


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

Dr.Said Alavi


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

Dr.Said Alavi

  • 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

Dr.Said Alavi

  • Hoffman 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

Dr.Said Alavi


Thank You

Dr.Said Alavi