• Introduction
• Symptoms
• Causes
• Diagnosis
• Treatment
• Complications
• Prevention
• Selected links
• Useful Article?

Introduction

Up until the middle of the twentieth century, rheumatic fever was a very common disease, particularly among children. The condition is now relatively rare in the developed world, largely due to significantly improved living conditions and the availability of antibiotics. However, in developing countries, rheumatic fever is still a major cause of death and heart disease.

Rheumatic fever develops after a streptococcal (strep) throat infection (a common group of bacterial infections). The exact cause of the condition is not yet known, but it tends to primarily affect the joints, skin and heart. Joints can become painful and swollen, patches and lumps (nodules) can appear on the skin and heart tissues, and heart valves can become damaged. However, most strep infections will not lead to rheumatic fever, even if the infection remains untreated.

Rheumatic fever is most prevalent in children between 5-15 years of age, although it can also develop in adults. Recurrent episodes of rheumatic fever are not uncommon, as being exposed to the disease does not provide immunity. Rheumatic fever usually reoccurs during the first five years following the initial infection.

There is no vaccine, or singular cure, for rheumatic fever, but 80% of patients with the condition make a full recovery after 12 weeks. The majority of patients will recover from their symptoms within this period, however, permanent damage to the heart sometimes occurs. Approximately 30-50% of patients will go on to develop rheumatic heart disease which can leave the heart valves damaged and unable to work to their full capacity.

Symptoms

Rheumatic fever often follows a streptococcal throat infection. In a third of streptococcal infections, there are few, or no, symptoms at all. Therefore, it is sometimes not until an individual goes on to develop rheumatic fever that the infection becomes apparent.

Symptoms of a streptococcal throat infection include:

• sore throat,
• red and swollen tonsils,
• fever,
• fatigue,
• headache, and
• muscle ache.

The symptoms of rheumatic fever usually appear between 1-5 weeks after a streptococcal throat infection. Some symptoms from the strep throat infection, such as muscle ache and headache, may worsen. Others will be replaced by new symptoms.

Symptoms of rheumatic fever include:

• fever,
• joint pain and swelling,
• abdominal pain,
• skin rashes or lumps (nodules),
• jerky body movements (chorea), and
• shortness of breath.

An attack of rheumatic fever may last for six weeks or longer.  Symptoms can be successfully treated, which often makes it difficult to be sure whether or not the fever has gone. However, there are various tests that can indicate whether or not the disease is active.

Heart damage may also occur as a result of rheumatic fever.  Often, the damage happens slowly, over a long period of time, and there may be few or no symptoms.  In the most severe cases this can lead to heart failure.

Causes

Rheumatic fever is not an infection. It starts following a strep throat caused by a particular strain of the bacteria called Streptococcus pyogenes, more commonly known as group-A haemolytic strep or strep A.

When streptococci get into the body, the immune system produces antibodies to attack them. Unfortunately, the antibodies also detect the similar identifying chemical groups on the body’s own tissues and attack them as well, causing inflammation and damage. If this happens, you will develop rheumatic fever.

The antibodies tend to attack the inner lining of the heart, the heart muscles, the joint lining surfaces, brain nerve tissue and other parts of the body.

Diagnosis

As rheumatic fever can take several different forms, there is no single test for the condition. A diagnosis of rheumatic fever is likely to be made from your symptoms and a physical examination focusing mainly on your heart, skin and joints.

The Jones Criteria
To identify rheumatic fever, your GP may use the Jones Criteria. They will check your symptoms against the criteria to determine whether or not it is likely that you have the condition. To be diagnosed with rheumatic fever, you must have at least two major criteria or one major and two minor criteria. The criteria are listed below:

Major criteria

• Carditis - inflammation of the heart
• Polyarthritis - where several joints become stiff, painful and swollen.
• Chorea - jerky involuntary body movements.
• Erythema marginatum - red or pink rash on the skin
• Subcutaneous nodules - small lumps under the skin that tend appear on the elbows, knees, ankles and knuckles.

Minor criteria

• Arthralgia - joint pain, but less severe than polyarthritis joint pain.
• Fever - usually over 39 º C (102 º F)
• Elevated ESR or CRP - erythrocyte sedimentation rate (ESR) and C reactive protein (CRP) are both types of blood tests that can detect inflammatory conditions. (see blood tests)
• Prolonged PR interval - irregular heart rhythm

Electrocardiogram (ECG)
To help confirm a diagnosis of rheumatic fever, you will usually be required to have an electrocardiogram (ECG). During an ECG up to 12 adhesive electrodes are attached to certain areas of the body, such as the arms, legs and chest. A machine will then measure the electrical activity of your heart, so that your doctor can check for any abnormal heart rhythms. Inflammation of the heart is a common complication of rheumatic fever, and so it is important that any abnormal heart rhythms are detected early on so that prompt treatment can be given.

Blood tests
A number of different blood tests may be used to look for indications of rheumatic fever. The first tests the level of C reactive protein in your blood. CRP is produced by the liver. If there is more CRP in the blood than usual then this means there is inflammation in the body.

Another blood test is known as erythrocyte sedimentation rate (ESR). In an ESR test a sample of your red blood cells are placed into a test tube of liquid. They are then timed to see how fast they fall to the bottom of the tube in millimetres per hour. If they are sinking faster than usual, this could mean that you have an inflammatory condition such as rheumatic fever

Treatment

In treating rheumatic fever, the main aims are to:

• eradicate the streptococcal infection,
• ease inflammation of the joints and skin, and
• provide supportive treatment for any cardiac (heart) problems.

The three main types of medicine that are used to treat rheumatic fever are:

• Antibiotics - penicillin is primarily prescribed to destroy any remaining streptococci bacteria. If you are allergic to penicillin, then an alternative antibiotic, such as erythromycin may be given. Children who develop rheumatic fever occasionally have to continue taking penicillin into their late teens to prevent a recurrence of the condition.
• Aspirin - may be prescribed to help ease and relieve joint pain. Children under 16 years of age should not take aspirin.
• Tranquillisers and sedatives - such as diazepam, are sometimes prescribed to help ease the symptoms of chorea. Chorea causes the body to move involuntarily.

If you have rheumatic fever, bed rest is essential, even if you are feeling well enough to be up and about. A patient should not leave their bed until there are no more indications of infection and inflammation. This can range from two weeks to three months, depending on the severity of the condition.

If you develop heart problems as a result of rheumatic fever, you may require surgery to repair, or replace, a damaged heart valve.

Complications

Rheumatic heart disease
About 30-50% of people who develop rheumatic fever will experience inflammation of the heart. It is therefore one of the most common, but also most serious, complications associated with rheumatic fever. Rheumatic fever can cause thickening and scarring of the heart valves, making them narrower (stenosis) or causing them to leak. It can also affect the pumping power of the heart, limiting its ability to efficiently circulate blood around the body.

This type of heart damage is known as rheumatic heart disease. If the damage is severe, surgery may be required to repair or replace the damaged valve, and prevent the disease from causing heart failure.

As well as heart disease, rheumatic fever can also cause other complications as outlined below.

• Joint problems - rheumatic fever can cause painful swelling and reddening of the joints.
• Nervous system - about 1 in 10 patients with rheumatic fever will experience an effect on their nervous system. The condition can lead to a loss of co-ordination and cause involuntary movements in the limbs and face. This is known as chorea or St Vitus' dance. However, it usually only lasts for a few weeks or months.
• Skin - occasionally rheumatic fever can cause irregular pink or red patches on your skin. You may also develop lumps or nodules under your skin. These are usually painless and range in size from a few millimetres to two centimetres. They tend to appear on the elbows, knees, ankles, knuckles and scalp. They will usually disappear within a month. ^^ Back to top

Prevention

Currently, rheumatic fever cannot be prevented, but research is underway to develop a vaccine which may be successful in preventing the condition in the future. Prompt treatment of a streptococcal throat infection with antibiotics will also help minimise the risk of the infection developing into rheumatic fever.

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