Rheumatic fever is an inflammatory disease that occurs in a very small percentage of children or adolescents with history of untreated strep throat infection. Symptoms of rheumatic fever generally appear a few weeks after the throat infection with group A beta-hemolytic streptococcus. There seems to be a genetic susceptibility to development of the disease, which is a body reaction to the streptococcus. There is no cure for rheumatic fever. It may be prevented by prompt and complete treatment of a strep throat infection with antibiotics. The disease may involve the heart, joints, central nervous system (brain), skin and subcutaneous tissue. Rheumatic fever usually occurs during the school-age years when strep throat infections are most prevalent. The incidence is low in most parts of the United States. The prevalence is higher in the colder months when strep throat is most likely to occur. Ninety percent of cases of rheumatic fever resolve in 3 months or less.
How it is diagnosed?
In 1944, the Jones criteria were formulated to make it easier to identify the disease. There are major and minor modified Jones criteria. In addition to evidence of a previous streptococcal infection, the diagnosis requires two major Jones criteria or one major plus two minor Jones criteria.
Major Jones Criteria
- Heart involvement
- A heart murmur is a common finding. This occurs in as many as 40% of patients and may include leaky valves, most commonly mitral regurgitation but also mitral and aortic insufficiency. In addition, the heart muscle and surrounding sac are affected as well. Patients develop unusually faster heart rates and may end up, although rarely, with congestive heart failure and accumulation of excessive amounts of fluid around the heart. Heart involvement is the major cause of long-term medical problems. Younger children tend to develop carditis (heart involvement) first. Patients with carditis are at a greater risk of developing recurrent rheumatic fever and also sustaining further heart damage. A significant percentage of patients with heart involvement end up with rheumatic heart disease (chronic heart involvement). Mitral stenosis is rare in the United States.
- Migratory poly-arthritis
- This condition occurs in 75% of patients and many times may be the initial clinical manifestations, especially in the older patients. It usually involves the large joints such as the knees, ankles, elbows and wrists. The term migratory means that it may start in only one knee and then gradually move to the contra-lateral knee joint. Joints become swollen, red and very tender. Joint motion is restricted and patients may have difficulty walking.
- Subcutaneous nodules
- They are firm, painless nodules on the extensor surface of the wrist, elbows and knees. They are found in only 10% of patients.
- Erythema Marginatum
- This skin rash occurs in over 5% of patients. The rash is serpiginous and may be long lasting or evanescent (tend to disappear and reappear).
- Sydenham Chorea
- It consists of rapid purposeless movements of the face and upper extremities. It is also called
St.Vitus Dance. Chorea movements are usually present when the patient is awake. Besides chorea there may be other clinical manifestations of brain involvement. Some children may develop mood swings and cry for no reason.
Minor Jones Criteria
- Previous history of rheumatic fever
- Arthralgia or joint pain (without arthritis)
- Prolongation of PR interval in the electrocardiogram (approximately 25% of all cases)
- Abnormal blood test results
Patients with rheumatic fever need to be treated with antibiotics regardless of a negative throat culture. High doses of aspirin or Naproxen are useful in controlling pain and inflammation. Steroids are rarely used except for extremely sick children, mainly patients in heart failure. Patients that develop heart failure will require heart medications and diuretics.
Secondary prophylaxis to prevent future strep infections is used in patients who develop acute rheumatic fever. The duration of prophylaxis depends on the risks of exposure to strep infections and if the patient had previous attacks of rheumatic fever. Penicillin is the drug of choice. Prophylaxis is usually given for at least five years (or to age 21) in those patients without heart involvement. Prophylaxis is given for a longer period of time if there has been heart involvement or chronic heart damage (rheumatic heart disease may require life-long prophylaxis).
Most patients do not require SBE prophylaxis under the new guidelines from the American Heart Association. A decision on whether or not to do this should be made after consultation with the family and Dr. Villafañe. Chorea movements may be controlled with medication as well.
Children's Heart Specialists, PSC is committed to provide comprehensive pediatric cardiac services to infants, children and young adults. We strive to insure the highest quality in non-invasive cardiovascular testing. Our medical staff is committed to being board certified in pediatric cardiology and adult congenital heart disease.
Our goal is to provide the highest quality of care with compassion and respect to our patients and their families.