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Rheumatic fever Content Supplied by NHS Choices
Introduction

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

The symptoms of rheumatic fever include joint pain and swelling (arthritis) and 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.

Rheumatic fever is rare in the UK.

What causes rheumatic fever

The symptoms of rheumatic fever aren't caused by the bacteria itself, but the immune system's response to it.

When your body senses the streptococcal infection, it sends antibodies (infection-fighting molecules) to fight it. However, the antibodies sometimes attack the tissues of parts of the body, such as the joints or heart instead. If the antibodies attack your heart, they can cause your heart valves to swell, which can lead to scarring of the valve "doors" (called leaflets or cusps).

Read more about the causes of rheumatic fever.

Treating rheumatic fever

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

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

Read more about treating rheumatic fever.

Complications

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

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

Read more about the complications of rheumatic fever.

Who's affected

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

The condition is now very rare in the UK as a result of higher standards of living and medical care. It's 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 five and 15. It's less likely to affect younger adults, and it's very rare for it to develop in adults who are 35 years of age or over. Both sexes are equally affected.

Outlook

The outlook for people with rheumatic fever depends on whether they've sustained significant heart damage.

If the heart is damaged, it's unlikely to fully recover. In such cases, 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 occurring again, which should hopefully prevent the heart becoming damaged.

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

Antibiotics
Antibiotics are medicines that can be used to treat infections caused by micro-organisms, usually bacteria or fungi. Examples of antibiotics include amoxicillin, streptomycin and erythromycin.
Bacteria
Bacteria are tiny, single-celled organisms that live in the body. Some can cause illness and disease and others are good for you.
Disease
A disease is an illness or condition that interferes with normal body functions.
Fever
A fever is when you have a high body temperature (over 38 C or 100.4 F).
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 of rheumatic fever, 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. Several joints are usually affected at the same time.

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

Heart inflammation (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.

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

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

Sydenham's chorea

Sydenham's chorea is a term that describes 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
  • difficulty with tasks requiring fine hand movements, such as writing
  • problems 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 rare in adults.

Sydenham's chorea usually passes within a few months, although in some cases it can persist for up to two years. It shouldn't cause any permanent damage to the nervous system.

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 children with fair skin.

The rash usually comes and goes over the course of a few weeks or months, before disappearing altogether.

It's 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 high temperature (fever) of 39C (102F) or above
  • abdominal pain
  • nosebleeds
Causes of rheumatic fever

Most experts believe rheumatic fever is caused by the immune system overreacting to the presence of group A streptococcus bacteria.

Almost all cases develop a few weeks after a throat infection with group A streptococcus bacteria.

During this throat infection, the lining of the throat becomes inflamed as the immune system responds to the infection.

In rheumatic fever, the process of inflammation seems to spread through the body in an uncontrolled way. The inflammation can affect the:

  • heart, which causes the symptoms of chest pain, fatigue and shortness of breath
  • joints, which causes the symptoms of arthritis
  • skin, which causes the skin rash and nodules to develop
  • nervous system, which causes the symptoms of chorea (uncontrollable jerking) and the changes in personality associated with rheumatic fever

It's not known why the immune system suddenly stops working properly. One theory is that the streptococcal bacteria have a similar molecular structure to certain tissues in the body. The immune system target="_blank"=""=""=""s not only the bacteria, but also tissues that share a similar molecular structure.

Another theory is that some people may be born with certain genetic traits that make their immune system more likely to malfunction after a throat infection.

Diagnosing rheumatic fever

As rheumatic fever can cause so many different symptoms, a 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 usually 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's chorea)
  • a painless, non-itchy skin rash (erythema marginatum)
  • bumps or lumps that develop underneath the skin (subcutaneous nodules)

The minor signs and symptoms are:

  • joint pain, but less severe than arthritic joint pain
  • a high temperature (fever), usually over 39C (102F)
  • blood tests that 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, or 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'll usually need to have an electrocardiogram (ECG). During an ECG, a number of small, sticky sensors called electrodes are attached to your arms, legs and chest. These are connected by wires to an ECG machine.

The ECG machine measures your heart's electrical activity, allowing your doctor to check for any abnormal heart rhythms. Heart inflammation is a common complication of rheumatic fever. It's important that any abnormal heart rhythms are detected early 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. These include:

  • C reactive protein (CRP) - which tests the level of C reactive protein (CRP) in your blood. CRP is produced by the liver. If there's more CRP in the blood than usual, there's 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, the red blood cells will settle higher up the tube. If these substances aren't present, the blood cells will be lower down the tube.
Treating rheumatic fever

If your child develops rheumatic fever, your GP will refer them to a paediatrician.

As rheumatic fever is very rare in England, they may also be referred to a doctor with experience of treating the 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 regular hospital visits may be needed so the person's heart can be closely monitored.

Treatment plan

There's currently no cure for rheumatic fever, but you can take steps to ensure your child feels as comfortable as possible and reduce the risk of serious complications. For example, it's possible to:

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 isn't usually recommended in children under the age of 16 because there's a small risk of it causing Reye's syndrome, a potentially fatal condition that can lead to liver and brain damage.

However, an exception is often made for rheumatic fever because most children only need to take a low-dose aspirin for one to two weeks, and it has proved very successful in relieving symptoms. Most healthcare professionals believe the benefits of using aspirin to treat rheumatic fever far outweigh the risks.

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

Prednisolone is usually given in tablet form as a course lasting two to six weeks. Side effects include headache, dizziness, problems sleeping and weight gain. These side effects should pass once the course is finished.

Antibiotics

It's 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 settling in the throat and causing another streptococcal throat infection. This is because further throat infections may lead to another episode of rheumatic fever.

Repeated episodes of rheumatic fever increase the risk of causing permanent heart damage. Antibiotic injections (intravenous or intramuscular) every two to three weeks over the course of many years are usually recommended.

The current recommendations state that if your child:

  • didn't experience any heart inflammation, the course should last for five years or until your child is 18 (whichever is longer)
  • experienced heart inflammation but their heart recovered, the course should last for 10 years or until your child is 25 (whichever is longer)
  • had heart inflammation that caused significant, persistent heart disease, the course should last until they're at least 40-45 (some doctors have recommended that the course should last for the rest of their life)

What if my child misses or forgets an injection

It's very important that your child doesn't miss an antibiotic injection. If they do, you must arrange for them to get the next injection as soon as possible.

Remember to tell your doctor or nurse if your child is going overseas, on holiday, away for a while, or you're moving house. The doctor or nurse will be able to arrange ongoing treatment for your child.

Your child should never stop antibiotic treatment without discussing it first with your doctor.

Will my child be able to lead a normal life

With proper care and regular antibiotic injections, most children with rheumatic fever are able to lead a normal life.

The important thing is to make sure your child never has another attack of rheumatic fever. The only way to do this is to make sure they have their regular antibiotic injections.

Bed rest

Plenty of bed rest is recommended because it will help reduce the strain on the heart and ease some of the symptoms, such as shortness of breath and persistent tiredness.

Your child can slowly start to increase their activity levels as they begin to recover. The doctor in charge of your child's care will be able to make more detailed recommendations.

Sydenham chorea

If your child has 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.

Medication may be recommended if the episodes are severe. Medicines originally designed to treat epilepsy, such as carbamazepine and valproic acid, are usually effective in treating Sydenham chorea.

However, 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 their dosage can be revised.

Complications of rheumatic fever

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

In rheumatic heart disease, inflammation causes the heart's valves to become damaged and stiffened, disrupting the normal flow of blood through the heart.

It's 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, which 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 can't 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.

Read more about treating heart failure.

 
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