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Rheumatic fever

Rheumatic fever is an inflammatory disease which may develop after a Group A streptococcal infection (such as strep throat or scarlet fever) and can involve the heart, joints, skin, and brain. more...

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General Information

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. In the Western countries, it became fairly rare since the 1950's, possibly due to higher hygienic standards. While it is far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a mortality of 2 - 5%.

Rheumatic fever primarily affects children between ages six and 15 and occurs approximately 20 days after strep throat or scarlet fever. In up to a third of cases, the underlying strep infection may not have caused any symptoms.

The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3 percent. The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode of rheumatic fever. Heart complications may be long-term and severe, particularly if the heart valves are involved.

Diagnosis: Modified Jones Criteria

T. Duckett Jones, MD first published these criteria in 1944. They have been periodically revised by the American Heart Association in collaboration with other groups. Two major criteria, or one major and two minor criteria, when there is also evidence of a previous strep infection support the diagnosis of rheumatic fever.

Major Criteria

  • Carditis: inflammation of the heart muscle which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
  • Migratory polyarthritis: a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
  • Sydenham's chorea (St. Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.
  • Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spread outward to form a snakelike ring while clearing in the middle. This rash never starts on the face and is made worse with heat.
  • Subcutaneous nodules (a form of Aschoff bodies): painless, firm collections of collagen fibers on the back of the wrist, the outside elbow, and the front of the knees. These now occur infrequently.


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Is rheumatic fever making a comeback? - streptococcal infections may evolve into rheumatic fever - Brief Article
From Science News, 11/28/98

A stealthy form of strep throat that doesn't cause symptoms may underlie a recent increase in the number of rheumatic fever cases seen at a Utah hospital. Primary Children's Hospital in Salt Lake City recorded 62 cases so far in 1998, a larger number than in any of the previous 38 full years. This tally kindles fears that a disease once on the decline may be reawakening.

Rheumatic fever, which can cause serious heart damage, evolves from an untreated Streptococcus A infection--strep throat. The sore throat is contagious but curable with penicillin. With antibiotic treatment of strep throats, deaths from rheumatic fever heart complications have fallen from about 15,000 in 1950 to 5,147 in 1995 nationwide. Many recent fatalities among the elderly stem from childhood rheumatic fever.

When scientists reviewed 478 cases of rheumatic fever treated between 1985 and 1998 at the Salt Lake City hospital, they found that only 20 percent of the patients had sought medical attention for throat problems before coming down with rheumatic fever symptoms. Nevertheless, more than 90 percent of these patients had manufactured antibodies against strep, says Lloyd Y. Tani, a pediatric cardiologist at the hospital, who presented the findings.

The deadliest complication of rheumatic fever is heart inflammation and is probably an autoimmune response. Doctors treat mild cases of heart inflammation with an anti-inflammatory drug, such as aspirin, and bed rest. In severe cases, they prescribe steroids. In this study, of the 297 patients whose rheumatic fever led to inflammation of the heart, 12 required surgery to repair damaged valves.

The best weapon against rheumatic fever is prevention, Tani says. If doctors see any signs of rheumatic fever in the community, they should start monitoring sore throats more carefully.

From a historical perspective, the new cases of rheumatic fever present a mystery, he says. Early in this century, health officials believed that crowded living conditions and poverty led to the spread of the strep bacteria. Most of the patients in the recent study, however, weren't considered poor.

COPYRIGHT 1998 Science Service, Inc.
COPYRIGHT 2000 Gale Group

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