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

Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. 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.

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. 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)
• Skin eruption on the trunk and upper part of arms or legs
• Eruptions that are ring-shaped or snake-like in appearance
• Skin nodules
• Sydenham's chorea
• Epistaxis
• Cardiac (heart) involvement which may be asymptomatic or may result in shortness of breath, chest pain

Signs

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, complete blood counts, and sedimentation rate. 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.

Major diagnostic criteria

These are also known as Jones criteria.

• Carditis (heart inflammation)
• Polyarthritis
• Subcutaneous skin nodules
• Chorea (Sydenham's chorea)
• Erythema marginatum

Minor diagnostic criteria

• Elevated erythrocyte sedimentation rate
• Elevated streptococcal antibody titer
• Prolonger PR interval on EKG
• Increased WBC count
• Elevated levels of C-reactive protein

To make a diagnosis

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.

Treatment

The management of acute rheumatic fever is geared toward 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

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.

Complications

• Damage to heart valves (in particular, mitral stenosis and aortic stenosis)
• Endocarditis
• Heart failure
• Arrhythmias
• Pericarditis
• Sydenham's chorea

External link

• NIH Rheumatic fever guide