Rheumatic Fever

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An inflammatory disease that may appear as a delayed reaction to inadequately treated strep throat, which can damage the heart. About 1 percent to 3 percent of untreated children come down with rheumatic fever from 10 days to six weeks after getting over a strep throat or SCARLET FEVER. Rheumatic fever usually appears in children between five and 18 and may affect the brain, joints, skin, or other tissues. Once diagnosed, the disease will tend to recur whenever the child gets a strep infection.

By the 1980s, the use of penicillin led to a dramatic drop in rheumatic fever in the United States. However, the disease has not been eradicated; today it involves a much more severe form that causes heart damage and death. Since 1985 there have been outbreaks in 24 states, especially Utah, Ohio, and Pennsylvania.

Researchers believe the resurgence can be traced to a reemergence of certain strains of bacteria (group A strep). Of the 80 different strains of group A strep, only a few can cause rheumatic fever. One of these strains of virulent bacteria killed Muppet creator Jim Henson in 1990.

Cause
Rheumatic fever is a delayed complication of group A strep infection, usually following either a strep throat or scarlet fever. It occurs because the patient’s body creates antibodies to get rid of toxins produced by the strep bacteria; in the process, those antibodies mistakenly attack the host’s own tissues in the joints, heart, skin, or nervous system. Scientists are not sure why some people develop rheumatic fever, but it may be a combination of the type of bacteria and the genetics of the infected person.

Symptoms
The onset of the disease is usually sudden, usually about one to eight weeks after recovery from scarlet fever or a sore throat. Early symptoms include fever, joint and muscle pain and swelling, nose bleeds, stomach pain, and vomiting. Joint pain usually affects the knees, hips, wrists, elbows, and shoulders and may move from one site to the next. Other symptoms include palpitations, chest pain, and heart failure.

Late signs of rheumatic fever are a clumsiness and awkwardness; as the symptoms progress, the child develops irregular body movements or twitching that can become severe, sometimes including the tongue and facial muscles. This group of symptoms is called “Sydenham’s chorea” or “St. Vitus’ Dance.” Fortunately, these movements do not last long and disappear without long-term damage.

Although most symptoms disappear within weeks to months, half the time the condition damages the heart valves. The mitral valve of the heart is most often affected, although the aortic valve also may be damaged. Occasionally, the heart muscle itself and the outside covering of the heart may become inflamed. In the most severe infections, heart failure may result. This severe heart damage most often occurs in children who have several attacks of rheumatic fever, which is why it is so important to prevent future repeated attacks with prophylactic antibiotics.

Diagnosis
Rheumatic fever is hard to diagnose because it resembles so many other illnesses; lab studies, throat culture, and an electrocardiogram can help. Doctors may use a variety of criteria, including evidence of previous group A strep infection.

A diagnosis of rheumatic fever requires one (and preferably two) of the following symptoms:

• joint pain
• clumsy movements lasting from three to eight months
• inflammation of the heart muscle
• painless swellings under the skin over the joints
• rash (flat and painless)
• fever of at least 100.4°F

Treatment
Severe restriction of regular activity, together with the administration of painkillers and penicillin to eliminate any remaining strep from the previous infection. Steroids or salicylates may be used depending on the severity of the joint pain. Antibiotic treatment may be continued daily for many years to prevent recurrences of rheumatic fever, which are most likely to occur during the first three years after the initial attack. Antibiotics are usually continued until the child reaches age 18. While bed rest used to be part of treatment, it is no longer considered to be helpful.

Complications
Heart inflammation occurs in about half of all patients and can lead to congestive heart failure. It is one of the most common causes of the need for heart valve replacement.
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