Rheumatic fever

Systemic immune sequelae of a β-hemolytic streptococcal infection of the pharynx.

Signs of RF occur 2-3 weeks after infection but may appear as early as 1 week or as late as 5 weeks.

Increasingly rare in the US.

Peak incidence 5-15 years of age, and rare before age 4 and after age 40 years.

Associated with a perivascular granulomatous reaction with vasculitis.

75 to 80% of cases involve the mitral valve, while the aortic valve is involved in 30% of cases, the tricuspid and pulmonary valves are involved in fewer than 5% of cases.

Major criteria for diagnosis includes carditis, erythema marginatum and subcutaneous nodules, Syndenham’s chorea, and polyarthritis.

Minor criteria include fever, polyarthralgias, reversible prolongation of the PR interval, elevated sedimentation rate or CRP.

The presence of two major criteria, all one major criteria and two minor criteria establishes the diagnosis.

Supporting diagnostic evidence includes a throat culture or rapid streptococcal antigen test and elevated or rising streptococcal antibody titers.

Carditis suggested by the presence of pericarditis, cardiomegaly, congestive heart failure, mitral or aortic regurgitation murmurs, or the presence of the Carey-Coombs short mid-diastolic mitral murmur, EKG changes including alterations in P waves, or T waves, and arrhythmic changes.

Long-term complications includes the development of rheumatic heart disease with valvular involvement affecting the mitral valve most commonly, the aortic valve is second most commonly involved and the tricuspid valve is the third most commonly involved.

Pulmonary valve involvement is rare.

Long-term complications include arrhythmias, pericarditis, and rheumatic pneumonitis.

Treatment includes bed rest until clinical symptoms abate and the sedimentation rate, resting pulse rate and EKG returned to baseline.

Penicillin is used to eradicate the infection.

The initial episode of rheumatic fever can be prevented by treatment of the streptococcal pharyngitis with penicillin.

Recurrent episodes of rheumatic fever must be prevented.

Rheumatic fever recurrences most common In patients who had carditiis associated with their initial course, and in children.

Children have a 20% chance of her recurrence within five years of experiencing rheumatic fever.

Prophylaxis is with benzathine penicillin 1.2 million units intramuscularly every four weeks or the use of oral penicillin.

Rheumatic fever recurrences are uncommon after five years and in patients over the age of 25.

Mortality rate 1 to 2%.

Persistent carditis is associated with a poor prognosis, with 30% of children so affected dying within 10 years.

Two thirds of patients will develop valvular abnormalities after 10 years.

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