ARF is a non-infective sequelae of pharyngitis caused by group A beta-hemolytic streptococcus. Symptoms usually appear two to three weeks after the initial infection. Although the incidence of ARF has declined dramatically after the introduction of antibiotics, the fall has only been modest. The changing epidemiology is most likely do to the change in the M protein of the streptococci leading to alterations in rheumatogenicity. Although the initial clinical syndrome may resolve, there may be devastating cardiac consequences from valvular damage. In the U.S., rheumatic heart disease is responsible for approximately 3500 deaths annually, compared to 90,000 deaths worldwide. There is equal incidence in boys and girls, and the median age of affected patients is 10 years.

Clinical features and diagnosis:

The diagnosis of ARF is made by applying the modified (1992) Jones criteria. Diagnosis requires evidence of recent streptococcal infection and 2 major criteria, or 1 major and 2 minor criteria

• Major criteria:
• Polyarthritis: Migrating arthritis that typically affects the knees, ankles, elbows and wrists. The joints are very painful and symptoms are very responsive to anti-inflammatory medicines.
• Pancarditis: All layers of cardiac tissue are affected (pericardium, epicardium, myocardium, endocardium) The patient may have a new or changing murmur, with mitral regurgitation being the most common followed by aortic insufficiency. . Mitral stenosis is rarely seen in the acute case, it instead indicated late scarring and calcification of a damaged valve.
• Chorea: Also known as Syndenham’s chorea, or "St. Vitus’ dance". There are abrupt, purposeless movements. This may be the ONLY manifestation of ARF and is its presence is diagnostic. May also include emotional disturbances and inappropriate behavior. Chorea is often misdiagnosed.
• Erythema marginatum: A non-pruritic rash that commonly affects the trunk and proximal extremities, but spares the face. The rash typically migrates from central areas to periphery, and has well-defined borders.
• Subcutaneous nodules: Usually located over bones or tendons, these nodules are painless and firm.
• Minor criteria:
• Arthralgia
• Fever
• Previous ARF or rheumatic heart disease
• Leukocytosis, Elevated ESR and CRP
• Prolonged P-R interval on EKG
• Evidence of recent streptococcal infection can be established by a positive throat culture, rapid strep test, or by an elevated or rising ASO titer, Anti-Deoxyribonuclease B, and Anti-Hyaluronidase. Often there is no history of a previous sore throat and the throat culture will be negative at the time of diagnosis.

1. Relieve acute symptoms with steroids and aspirin

2. Eradicate group A beta-hemolytic streptococcus

3. Chorea- may get symptomatic relief with Sodium Valproate or Haloperidole

3. Prophylaxis against future infection to avoid recurrent cardiac disease

a. No carditis- prophylaxis for 5 years or until 21 years old

b. Carditis without residual cardiac defect- prophylaxis for 10 years

c. Cardiac defect-prophylaxis until 40 or later.

Reference

1. Mirkinson Laura. The Diagnosis of Rheumatic Fever. Pediatrics in Review September 1998

2. Alseid Khaled. And Majeed H.A. Acute Rheumatic Fever: Diagnosis and Treatment. Pediatric Annals May 1998

3. Revised Jones Criteria  JAMA 1992; 269: 2069-2073

4. Zamorradi A. Wald E. Syndenham's Chorea in Western Pennsylvania.  Pediatrics April 2006

5. American Heart Association Guidelines.  Prevention of Endocarditis.  Circulation April 19, 2007