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Rheumatic fever

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Rheumatic fever



Introduction 

Rheumatic fever is a serious complication that can develop following an untreated throat infection (by a type of bacteria called group A streptococcus).

Rheumatic fever is uncommon in the UK.

Symptoms include:


joint pain and swelling (arthritis)

inflammation of the heart, which can cause shortness of breath and chest pain


Symptoms usually last around four weeks, but can sometimes persist for several months.

What causes rheumatic fever?

The symptoms of rheumatic fever are not caused by the bacteria itself, but the immune system’s response to the bacteria.

The immune system, which is the body’s defence against infection, mistakes healthy tissue for the streptococcus bacteria and causes parts of the body to become inflamed (swollen and filled with fluid). This triggers the symptoms of rheumatic fever.

Treatment

There is currently no cure for rheumatic fever. Treatment involves relieving symptoms with medication and trying to prevent permanent damage to the body, especially the heart.

Once a person has had an attack of rheumatic fever, it is very common for them to have future attacks. This can be prevented by taking a long-term course of antibiotics.

Complications

Rheumatic fever can cause permanent damage to the valves of the heart; this is known as rheumatic heart disease.

Rheumatic heart disease can lead to serious complications, including heart failure and stroke.

Who is affected

Rheumatic fever is very common in poorer parts of the world, such as Africa, the Middle East and South America, where there is over-crowding, poor sanitation and limited access to medical treatment. It is estimated that just under half a million new cases of rheumatic fever occur worldwide each year.

The condition is now extremely rare in the UK, due to higher standards of living and medical care. It is estimated that less than 1 in 100,000 people in the UK develop rheumatic fever.

Most cases of rheumatic fever first develop in children between the ages of 5 and 15. It becomes less widespread in younger adults, and it is very rare for it to develop in adults aged 35 or over. Both sexes are equally affected.

Outlook

The outlook for people with rheumatic fever depends on whether they have sustained significant damage to the heart.

If the heart is damaged, it is unlikely to fully recover. Should this happen, the symptoms of rheumatic heart disease, such as shortness of breath and constant tiredness, will continue.

If the heart is undamaged, long-term use of antibiotics should prevent rheumatic fever from occurring again, hopefully preventing further damage to the heart.

Deaths associated with rheumatic heart disease are very rare in the UK and the rest of the developed world.

Symptoms of rheumatic fever 

The symptoms of rheumatic fever usually develop one to five weeks after a streptococcal throat infection.

Common symptoms

Common symptoms of rheumatic fever are described below.

Arthritis

Pain and swelling of the joints (arthritis) is the most common symptom, affecting three out of four people.

The larger joints, such as the knees, ankles, elbows and wrists are usually affected, typically on both sides of the body. Normally, several joints are affected at the same time.

The symptoms of arthritis should pass within four weeks as the inflammation settles, without causing any permanent damage.

Inflammation of the heart (carditis)

Inflammation of the heart (carditis) is another common and potentially serious symptom of rheumatic fever.

Carditis occurs in an estimated 30-60% of people with rheumatic fever and is more common in younger children.

Because of the inflammation, the heart has difficulty pumping blood around the body, which can cause the following symptoms:


shortness of breath, especially when a person is physically active or when sleeping (this can often wake a person up, gasping for breath)

persistent cough

rapid heartbeat (tachycardia)

feeling tired all the time

chest pain


Carditis can persist for several months, but it should improve over time.

Sydenham's chorea

Sydenham's chorea is a term used to describe a collection of symptoms related to inflammation of the nerves. These symptoms are:


involuntary and uncontrollable jerking and twitching of the body – most often, the hands and feet

difficulties with tasks requiring fine hand movements, such as writing

difficulties with balance

unusual emotional outbursts, such as crying or laughing for no apparent reason


Around in one in four children with rheumatic fever will develop Sydenham's chorea, but it is very rare in adults.

Sydenham's chorea usually passes within a few months and should not cause any permanent damage to the nervous system. However, there have been some reports of it persisting for up to two years.

Skin rash

Around 1 in 10 children with rheumatic fever will develop a skin rash, known as erythema marginatum. The rash is usually painless, non-itchy and spreads slowly over the child’s body. It may only be noticeable in fair-skinned children.

The rash will normally come and go over a few weeks or months, before going away altogether.

It is very rare for adults with rheumatic fever to develop a skin rash.

Less common symptoms

Less common symptoms of rheumatic fever include:


subcutaneous skin nodules – small painless bumps or lumps under the skin, usually found on the wrists, elbows and knees

a very high temperature (fever) of 39°C (102°F) or above

abdominal pain

nosebleeds


Diagnosing rheumatic fever 

As rheumatic fever can cause so many different symptoms, a type of checklist known as the "Jones Criteria" is used to help diagnose it.

Your GP will use the Jones Criteria to check for the signs and symptoms strongly associated with rheumatic fever.

These are divided into:


major signs and symptoms – which you would normally expect to see in a case of rheumatic fever

minor signs and symptoms – which can sometimes occur in a case of rheumatic fever


The major signs and symptoms are:


inflammation of the heart (carditis) – which can cause symptoms such as shortness of breath and chest pain

pain and swelling (arthritis) affecting multiple joints

jerky involuntary body movements and emotional outbursts (Sydenham chorea)

a painless, non-itchy skin rash (erythema marginatum)

bumps or lumps developing underneath the skin (subcutaneous nodules)


The minor signs and symptoms are:


joint pain, but less severe than arthritis joint pain

a high temperature (fever), usually over 39ºC (102ºF)

blood tests show you have high levels of inflammation in your body

an irregular heart rhythm


A confident diagnosis of rheumatic fever can usually be made if:


at least two major signs and symptoms are present

there are two minor, and one or more other major, signs or symptoms


Testing

While some of the signs and symptoms listed above can be assessed by a physical examination, others – such as inflammation of the heart – need to be tested. Tests used to diagnose rheumatic fever are outlined below.

Electrocardiogram (ECG)

You will usually need to have an electrocardiogram (ECG). During an ECG, up to 12 sticky pads containing electrodes are attached to certain areas of the body, such as the arms, legs and chest. This procedure is painless.

A machine then measures 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. 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 also be used to look for indications of rheumatic fever.


C reactive protein (CRP) – this tests the level of C reactive protein (CRP) in your blood. CRP is produced by the liver. If there is more CRP in the blood than usual, there is inflammation in the body.

Antistreptolysin O titre (ASOT) – this blood test looks for evidence of antibodies produced by the immune system in response to the streptococcal infection.

Erythrocyte sedimentation rate (ESR) – in an ESR test, a sample of your red blood cells is placed into a test tube of liquid. If the blood is "sticky" due to various substances produced during the immune response, then the red blood cells will settle higher up the tube when it stands up. If these substances are not present, the ESR will be lower.


Treating rheumatic fever 

If your child develops rheumatic fever, they will be referred to a paediatrician by your GP.

Because of the very rare nature of rheumatic fever in England, they may also be referred to a doctor with previous experience of treating this condition so a treatment plan can be drawn up.

This may involve visiting a hospital or specialist clinic in one of the larger cities in England.

Most people with rheumatic fever are well enough to be treated at home, but they may need to make regular hospital visits so the state of their heart can be monitored.

Treatment plan

There is currently no cure for rheumatic fever, but it is possible to make your child feel as comfortable as possible while reducing the risk of serious complications.

This can be achieved by:

Anti-inflammatory medications

Anti-inflammatory medications can be used to relieve symptoms of joint pain, swelling (arthritis) and, in severe cases, reduce inflammation of the heart.

Non-steroidal anti-inflammatory drugs (NSAIDs), which include painkillers such as ibuprofen, are commonly used to relieve arthritis.

The use of aspirin is not usually recommended in children under the age of 16 as there is a very small risk of causing Reye’s syndrome – a potentially fatal condition that can cause liver and brain damage.

However, an exception is usually made for rheumatic fever, as most children are only required to take a low-dose aspirin for one to two weeks, and it has proved extremely successful in relieving symptoms. Most health professionals believe the benefits of aspirin in the treatment of rheumatic fever far outweigh the risks.

If the results of your electrocardiogram (ECG) show inflammation of the heart, a type of steroid medication called prednisolone will usually be recommended.

Prednisolone is usually given in tablet form for a course of two to six weeks.

Side effects of prednisolone include headache, dizziness, problems sleeping and weight gain.

These side effects should pass once the course is finished. 

Antibiotics

It is important to get rid of any streptococcus bacteria left in your child’s body from the infection. It's also important to prevent any streptococcus bacteria picked up after the initial infection from settling in the throat and causing another infection (known as prophylaxis). This is because further throat infections may lead to another episode of rheumatic fever.

Repeated episodes of rheumatic fever increases the risk of causing permanent damage to the heart.

Injections of antibiotics (intravenous antibiotics) every two to three weeks over the course of many years is usually recommended.

The current recommendations are:

Bed rest

Plenty of bed rest is recommended, as this will help reduce the strain on the heart and help ease some of the symptoms, such as shortness of breath and constantly feeling tired.

As your child begins to recover, they can slowly increase their activity levels.

The doctor in charge of your child’s care will be able to make more detailed recommendations.

Sydenham chorea

If your child is experiencing episodes of Sydenham chorea (uncontrollable physical jerking and emotional outbursts), moving them into a calm, quiet environment, such as a darkened bedroom, can help improve their symptoms.

If these episodes are more severe, medication may be recommended. Medicines originally designed to treat epilepsy, such as carbamazepine and valproic acid, are usually effective in treating Sydenham chorea.

If the dose is too high, these medicines can cause side effects similar to being drunk, including dizziness, double vision and vomiting.  

If your child experiences any of these symptoms, contact the doctor in charge of their care so that the dosage can be revised.

Page last reviewed: 24/02/2014

Next review due: 26/02/2016

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Complications of rheumatic fever 

Rheumatic heart disease is a common and potentially serious complication that can arise in cases of rheumatic fever.

Rheumatic heart disease develops when inflammation causes the valves in the heart to become damaged and stiffened, meaning that the normal flow of blood through the heart is disrupted.

It is estimated that around one in three people with a history of rheumatic fever will go on to develop rheumatic heart disease.

Symptoms of rheumatic heart disease include:

It can take many years for these symptoms to develop after a previous episode of rheumatic fever.

Mild rheumatic heart disease can usually be treated with medication, such as ACE inhibitors. ACE inhibitors relax your arteries, making it easier for your heart to pump blood around your body.

Atrial fibrillation

In some cases, rheumatic heart disease can lead to atrial fibrillation, a heart condition that causes an irregular and often abnormally fast heart rate.

Atrial fibrillation can lead to an increased risk of a stroke. Treatment may involve medication to control the heart rate or rhythm, and medication to prevent a stroke.

Heart failure

In more severe cases of rheumatic heart disease, the heart becomes so damaged that it cannot pump enough blood around the body. This is known as heart failure.

Heart failure that occurs in people with rheumatic heart disease may require surgery, either to replace a damaged valve with an artificial one or expand the valve with a tiny balloon.

Endocarditis prophylaxis

If you have had rheumatic fever before, you may need antibiotics during surgery to protect the heart valves from becoming infected (endocarditis).


using anti-inflammatory medications to relieve symptoms 

using antibiotics to clear out any remaining streptococcus bacteria in your child’s body

plenty of bed rest

if your child did not experience any inflammation of the heart, the course should last for five years or until your child is 18 (whichever is longer)

if your child did experience inflammation of the heart but their heart recovered, the course should last for 10 years or until your child is 25 (whichever is longer)

if your child had inflammation of the heart that caused significant, persistent heart disease, the course should last until they are at least 40-45 (some doctors have recommended that the course should last for the rest of their life)

dizziness

chest pain

shortness of breath

tiredness

Rheumatic-fever