Rheumatic fever

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

Introduction

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

The symptoms of rheumatic fever include:

  • joint pain and swelling (arthritis)
  • inflammation of the heart, which can cause shortness of breath and chest pain
  • fever

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

Read more about the symptoms of rheumatic fever.

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, which causes parts of the body to become inflamed (swollen and filled with fluid). This triggers the symptoms of rheumatic fever.

Read more about the causes of rheumatic fever.

Treatment

There is currently no cure for rheumatic fever so 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 further attacks in the future. 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 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.

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 experienced significant damage to the heart.

If the heart is damaged then it is unlikely to recover fully. In this case, the symptoms of rheumatic heart disease, such as shortness of breath and feeling tired all the time, will continue.

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

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 are tiny, single-celled organisms that live in the body. Some can cause illness and disease and others are good for you.

A disease is an illness or condition that interferes with normal body functions.

A fever is when you have a high body temperature (over 38°C or 100.4°F).

Symptoms

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 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 is thought to occur in an estimated 30-60% of cases and is most common in younger children.

Due to the inflammation, the heart has difficulties 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
  • feeling tired all the time
  • chest pain

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

Sydenham chorea

Sydenham 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 chorea, but it is very rare in adults.

Sydenham chorea usually passes within a few months and should not cause any permanent damage to the nervous system. Although 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. 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 the course of a few weeks or months, before going away altogether.

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

Less common symptoms

Less common symptoms of rheumatic fever include:

  • small painless bumps or lumps under the skin
  • a very high temperature (fever) of 39°C (102°F) or above
  • abdominal pain
  • nosebleeds

Causes

Most experts believe rheumatic fever is caused by the immune system malfunctioning. Almost all cases develop after a throat infection with streptococcus bacteria.

During a throat infection, the lining of your throat becomes inflamed to prevent the infection from spreading. This is caused by your immune system responding to the infection.

In cases of rheumatic fever, it seems that the process of inflammation spreads throughout your body in an uncontrolled way. The inflammation can affect:

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

Exactly why the immune system suddenly stops working properly is unclear. One theory is that the streptococcal bacteria have a similar molecular structure to certain tissues in the body. So the immune system may begin by targeting the bacteria and then mistakenly go on to target tissues that share a similar molecular structure.

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

Diagnosis

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 doctor will use the Jones Criteria to check whether you have 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
  • bumps or lumps developing underneath the skin

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, or
  • there are two minor signs and symptoms and at least one major sign or symptom

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 adhesive electrodes are attached to certain areas of the body, such as the arms, legs and chest.

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, 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 also be used to look for indications of rheumatic fever. The first 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 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 is placed into a test tube of liquid. They are then timed to see how fast they fall to the bottom of the tube. If they are sinking faster than usual, this could mean that you have an inflammatory condition such as rheumatic fever.

Treatment

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

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 and swelling (arthritis) and, in severe cases, reduce inflammation of the heart.

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

The use of aspirin is not usually recommended in children under the age of 16 as it carries 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 in cases of 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 feel that 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. This is because if they develop further throat infections these may lead to another episode of rheumatic fever.

Repeated episodes of rheumatic fever carry an increased risk of causing permanent damage to the heart.

It is usually recommended that they are given injections of antibiotics (intravenous antibiotics) every two to three weeks over the course of many years.

The current recommendations are:

  • 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)

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 feeling tired all the time.

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.

Complications

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:

  • dizziness
  • chest pain
  • shortness of breath
  • tiredness

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.

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.

Read more about the treatment of heart failure.

Content supplied by NHS Choices