A systemic inflammatory disease which may develop after an infection with streptococcus bacteria (such as strep throat or scarlet fever ) and can involve the heart, joints, skin, and brain.
Causes, incidence, and risk factors
Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. While it is far less common in the U.S. since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Rheumatic fever affects primarily children between 6-15 and occurs approximately 20 days (usually within 1 to 5 weeks) after strep throat or scarlet fever . In up to a third of cases, the underlying streptococcal infection may not have caused any symptoms. The rate of development of acute rheumatic fever in individuals with untreated streptococcal infection is estimated to be 3%. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated streptococcal infections.
Signs and tests
Given the different manifestations of this disease, there is no specific test which can definitively establish a diagnosis. In addition to a careful physical examination with attention to heart sounds, skin, joints, blood samples may be taken as part of the evaluation. These include tests for recurrent strep infection ( ASO or antiDNAse B), complete blood counts and sedimentation rate ( ESR). As part of the cardiac evaluation, an electrocardiogram may also be done. In order to standardize the diagnosis of rheumatic fever, several minor and major criteria have been developed, which in conjunction with evidence of recent streptococcal infection allow a diagnosis of rheumatic fever. The major diagnostic criteria include:
carditis (heart inflammation) polyarthritis subcutaneous skin nodules
chorea (Sydenham's chorea) erythema marginatum. The minor criteria include fever, arthralgia, elevated erythrocyte sedimentation rate and other laboratory findings. Two major criteria, or one major and two minor criteria in the setting of evidence of a previous streptococcal infection (positive culture or rising antibody level -ASO or antiDNAse B) support the diagnosis of rheumatic fever.
Treatment
The management of acute rheumatic fever is geared towards the reduction of inflammation with anti-inflammatory medications such as aspirin and/or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics. Another important cornerstone in treating rheumatic fever includes the continuous use of low dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.
Expectations (prognosis)
The recurrence of rheumatic fever is relatively common in the absence of "maintenance" on low dose antibiotics especially during the first 3 to 5 years after the first episode of rheumatic fever. Heart complications may be long-term and severe, particularly if the heart valves are involved.
Complications
damage to heart valves (in particular, mitral stenosis and aortic stenosis)
endocarditisheart failurearrhythmiaspericarditis Sydenham's chorea
Calling your health care provider
Call your health care provider if you develop symptoms of rheumatic fever. There are numerous conditions which may have similar symptoms and so you will therefore require a careful medical evaluation. Also, if you have symptoms of strep throat, notify your health care provider as you will need to be evaluated and treated if strep throat is confirmed to decrease your risk of developing rheumatic fever.
Prevention
The most important way to prevent rheumatic fever is by proper and prompt treatment of strep throat and scarlet fever.