Rheumatic fever

Alternative names 
Acute rheumatic fever

Definition
Rheumatic fever is an 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 primarily affects children between ages 6 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%. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

Symptoms

     
  • Fever  
  • Joint pain, migratory arthritis - involving primarily knees, elbows, ankles, and wrists  
  • Joint swelling; redness or warmth  
  • Abdominal pain  
  • Skin rash (erythema marginatum)       o Skin eruption on the trunk and upper part of arms or legs       o Eruptions that are ring-shaped or snake-like in appearance  
  • Skin nodules  
  • Sydenham’s chorea - emotional instability, muscular weakness and rapid, uncoordinated jerky movements affecting primarily the face, feet and hands  
  • Epistaxis (nosebleeds)  
  • Cardiac (heart) involvement which may be asymptomatic or may result in Shortness of breath, chest pain

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 of heart sounds, skin, and 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. These criteria, in conjunction with evidence of recent streptococcal infection, establish 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 (Joint pain), elevated erythrocyte sedimentation rate, and other laboratory findings.

Two major criteria, or one major and two minor criteria, when there is also evidence of a previous strep 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 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 of 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)  
  • Endocarditis  
  • Heart failure  
  • Arrhythmias  
  • Pericarditis  
  • 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, therefore you will require careful medical evaluation.

If you have symptoms of strep throat, notify your health care provider. 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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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