Adult rheumatic fever, juvenile rheumatic fever
What is rheumatic fever?
Rheumatic fever is a rare, serious immune disease that can cause inflammation and damage to several parts of the body, such as the heart, joints and nervous system.
What causes rheumatic fever?
The exact cause of rheumatic fever is not yet known, but it occurs shortly after (usually within one to five weeks) an infection with untreated group A streptococcus, the bacterium that causes "strep throat" and scarlet fever.
In some people, it appears that the body's immune system becomes over-active in its response to the streptococcus bacterium. This over-activity leads to inflammation and tissue damage in other parts of the body, particularly the heart.
Who gets rheumatic fever and who is at risk?
Rheumatic fever can occur at any age, but it is most common between the ages of five and 15 years.
Rheumatic fever is now uncommon in developed countries compared to 40 years ago, before the widespread use of the antibiotic penicillin. The disease is however still very common in developing countries such as South Africa.
Only a small percentage of people with untreated strep throat (approximately 3%) will develop rheumatic fever.
Symptoms and signs of rheumatic fever
Symptoms usually appear about two to four weeks after an untreated streptococcal infection, and may include:
- Joint pain, which often moves from joint to joint
- Joint swelling, which may be accompanied by redness and a sensation of heat
- Abdominal pain
- Skin rash: broad, pink to light-red patches that increase in size and do not itch
- Small lumps under otherwise normal-looking skin
- Unusual, involuntary jerky movements
- Muscle pain
- Decreased muscle tone
- Shakiness of one or more parts of the body
- Speech difficulties
- Fatigue and weakness
- Heart palpitations
- Chest pain
How is rheumatic fever diagnosed?
Your doctor will ask symptoms and whether you know or suspect you have had a recent streptococcal infection. He or she will probably also perform a physical examination, giving particular attention to listening for abnormal heart rhythms or murmurs, checking the joints for pain and inflammation, and examining the skin for rashes or lumps.
Doctors base a diagnosis of rheumatic fever on evidence of a preceding streptococcal infection, as well as the presence of at least two of the following major criteria, or at least one major criterion and two minor criteria:
- Inflammation of the heart (myocarditis), sometimes indicated by weakness, shortness of breath or chest pain. Heart inflammation may be suspected after a physical examination or in medical tests such as electrocardiogram or chest X-ray.
- Painful arthritis, most often affecting the joints of the ankles, wrists, knees and elbows, and migrating from joint to joint (migratory polyarthritis).
- Loss of co-ordination and involuntary, jerky movement of the limbs and face, or more subtle movement abnormalities, such as difficulties with handwriting. This symptom is known as Sydenham's chorea or Saint Vitus' dance.
- Pink or light-red patches on the skin, called erythema marginatum (uncommon).
- Subcutaneous skin nodules - lumps under the skin (uncommon).
- Joint pain without inflammation
- Previous rheumatic fever or evidence of rheumatic heart disease (permanent heart damage due to rheumatic fever)
- Abnormal heartbeat shown on an electrocardiogram
- Blood test indicating inflammation
- New heart murmurs
Although laboratory tests cannot confirm rheumatic fever, your doctor may recommend certain tests and procedures to help diagnose or investigate possible complications. Tests may include a throat culture of streptococcus (the throat is swabbed and the bacteria, if present, are grown in the laboratory). However, it is possible that by the time you see your doctor, your throat culture will be negative for streptococcus. Your doctor may also take a blood sample to test for the presence of antibodies (specific proteins the immune system produces in response to invasive organisms) to streptococcal bacteria.
A chest X-ray will often be taken, and your doctor may recommend an electrocardiogram of your heart to check for abnormal rhythms that indicate heart inflammation. This involves attaching electrode patches to your skin to measure the electrical impulses that your heart produces and which allow the heart muscles to contract. The impulses are transmitted to an electrocardiograph (ECG) machine, which records and represents them graphically as wave forms on a monitor or printout.
How is rheumatic fever treated?
The primary goal of treatment for rheumatic fever is to cure the infection and so help prevent complications. However, many patients will have recovered from the initial streptococcal throat infection by the time they see a doctor. Antibiotics are still prescribed to prevent a recurrence of the throat infection, and thus to prevent recurrence of the rheumatic fever. In addition, other medications are used to relieve symptoms of the disease.
Once rheumatic fever has developed, antibiotics will not be effective in stopping the course of the disease or preventing damage to the heart. Even so, your doctor may prescribe an antibiotic such as penicillin or erythromycin to fight the infection and destroy any remaining streptococcus bacteria. Side-effects of antibiotics may include stomach upset, nausea and diarrhoea, and allergic reactions in some people.
Medication to relieve symptoms of pain, swelling and fever include anti-inflammatory drugs (nonsteroidal anti-inflammatory drugs or NSAIDS, and corticosteroids), such as ibuprofen, and pain relievers such as aspirin or acetaminophen. Aspirin should not be used for children because of the risk of developing Reyes syndrome. Anti-inflammatory drugs can also sometimes prevent more severe tissue damage. Severe heart inflammation is usually treated with a corticosteroid medication, such as prednisone.
Hospitalisation for acute rheumatic fever may be necessary, and bed-rest until all signs of the active disease have disappeared is extremely important. This should be followed by a gradual increase in physical activity. You will also be encouraged to take fluids - at least six to eight glasses per day.
After you have recovered from acute rheumatic fever, you will still need to take prophylactic (preventative) antibiotics in the long term (usually to at least the early 20's) to protect against new streptococcal infections, which can trigger a recurrence of rheumatic fever, with risk of further organ damage. People with chronic or resistant rheumatic fever will need to stay on a schedule of antibiotics for several years, sometimes even for life, to prevent recurrences. Prophylactic antibiotics should also be given before and after dental or surgical procedures, to decrease risk of bacterial infection which can affect the damaged heart. If you have had rheumatic fever at any stage in your life, it is very important to tell your doctor or dentist if you are planning on having any sort of surgical or dental procedure done (even if it seems like a minor procedure). Your doctor or dentist will know which procedures are associated with a higher risk of subsequent infection on the heart, and give you prophylactic antibiotics if necessary.
Your doctor will continue to monitor you for complications of rheumatic fever, which will require additional special treatment. Monitoring may require repeated electrocardiograms to check heart valves, and urine tests to check for protein in the urine. Your doctor will also monitor side-effects of any medications used in treatment.
In cases of severe rheumatic heart disease, surgical repair or replacement of the heart valves may be necessary.
What is the outcome of rheumatic fever?
Most symptoms of rheumatic fever resolve after several weeks or months, but recurrence is common, and heart and joint complications may be long-term and severe.
The possible complications of rheumatic fever may include the following:
- Heart valve damage. Between 40% and 60% of patients develop heart inflammation with their first attack of rheumatic fever, and this often results in permanent scarring of the heart valves. A scarred heart valve may prevent adequate blood flow or cause backward flow of blood. Damage to the heart valves may show up 10 to 30 years after the initial infection.
- Endocarditis, an inflammation of the lining of the heart
- Heart failure as a result of inflammation of the heart muscle
- Arrhythmias or abnormal heart rhythms
- Pericarditis, an inflammation of the sac surrounding the heart
- Chronic skin disorders and sores
- Proteinuria, a loss of protein in the urine that may be associated with kidney damage
- Sydenham's chorea. Chorea usually subsides or disappears within weeks to months
- Chronically enlarged lymph nodes
Can rheumatic fever be prevented?
Prompt and effective treatment of streptococcal infection with antibiotics will usually prevent rheumatic fever developing, and also reduces the risk of streptococcus being transmitted to other people.
There are many different types of streptococcal infection, and not all of these cause rheumatic fever. However, some can cause kidney problems and other complications if left untreated.
When to call the doctor
Consult your doctor without delay if you have any symptoms of rheumatic fever.
Sore throat is usually caused by a virus infection, which does not lead to rheumatic fever and cannot be treated with antibiotics. However, if you have a sore throat together with a fever that has lasted for more than 24 hours, or a severe sore throat without much fever (especially if you have been in close contact with someone with strep throat), you should see your doctor to determine whether you have a streptococcal infection.
Reviewed by Dr Andrew Whitelaw, MBBCh (Witwatersrand), MSc (UCT), FCPath (Micro) (SA) Senior registrar, Department of Microbiology, University of Cape Town and Groote Schuur Hospital.