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Rheumatic fever

Overview

Rheumatic fever is a condition that can cause wide spread inflammation throughout the body. Symptoms of rheumatic fever include joint pain, skin rashes and jerky body movements.

Rheumatic fever develops as a complication of a type of bacterial throat infection known as a streptococcal infection (also known as ‘strep throat’).

Rheumatic fever can cause permanent damage to the valves of the heart, which is known as rheumatic heart disease. Rheumatic heart disease can cause potentially serious complications, such as heart failure, stroke and death.

While there is no cure for rheumatic fever, the symptoms can be relieved using a combination of antibiotics, anti-inflammatory medications and anticonvulsants (used to control the jerky body movements).

Complications
 

Rheumatic heart disease

Rheumatic heart disease is both the most common and the most serious complication that can arise in cases of rheumatic fever, occurring in an estimated 9-34% of cases.

Rheumatic heart disease develops when high levels of inflammation lead to the valves in the heart becoming damaged and stiffened, meaning that the normal flow of blood through the heart has been disrupted.

Symptoms of rheumatic heart disease include:

  • dizziness,
  • chest pain,
  • shortness of breath, and
  • tiredness.

Mild to moderate 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.

Moderate to severe rheumatic heart disease may require surgery. For example, damaged valves can be removed and then replaced with artificial valves.

Causes

The exact cause of rheumatic fever is uncertain. As almost all cases develop after a throat infection, most experts believe the condition is caused by your immune system malfunctioning.

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

In cases of rheumatic fever, it seems that the process of inflammation goes out of control and spreads throughout your body, including:

  • your heart, which causes the symptoms of chest pain, fatigue and shortness of breath,
  • your joints, which causes the symptoms of arthritis,
  • your skin, which causes the symptoms of the skin rash and nodules, and
  • your 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 associated theory is that certain 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 in the diagnosis process.

The Jones Criteria involves checking whether you have certain signs and symptoms strongly associated with rheumatic fevers. These signs and symptoms are collectively known as criteria.

There are two types of criteria:

  • major criteria, which are signs and symptoms strongly associated with rheumatic fever, and
  • minor criteria, which are signs and symptoms moderately associated with rheumatic fever.

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 arthritis joint pain.
  • Fever: usually over 39ºC (102ºF).
  • 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 below).
  • Prolonged PR interval: irregular heart rhythm.

A confident diagnosis of rheumatic fever can be made if you have at least two major criteria or one major and two minor criteria.

Testing

While some of the criteria listed above can be assessed by a physical examination and asking you (or your child) about your symptoms, others, such as inflammation of the heart, will require testing to confirm. Tests used in the diagnosis of rheumatic fever are outlined below.

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. If they are sinking faster than usual, this could mean that you have an inflammatory condition such as rheumatic fever.

Treatment

Rheumatic fever is usually treated in hospital, so that your heart can be carefully monitored.

Treatment goals

There is no cure for rheumatic fever so treatment focuses on three goals:

  • to eradicate any streptococcal bacteria that may be remaining in your body with antibiotics,
  • to help relieve symptoms with anti-inflammatory medication, and
  • to prevent long-term damage to your heart.

Antibiotics

The reason it is important to get rid of any streptococcal bacteria left in your body is that if you then experience another throat infection you may also experience 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 you are given injections of antibiotics (intravenous antibiotics) every two to three weeks over the course of many years. In cases of children, it is usually recommended that they are treated with antibiotics until they reach adulthood.

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.

Common type of anti-inflammatory medications used to relieve arthritis include the non-steroidal anti-inflammatory drugs (NSAIDs) type of painkillers such as ibuprofen, and aspirin.

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. So 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 that you have moderate to severe inflammation of the heart, you will probably be treated with a type of steroid medication called prednisolone.

 

Prednisoloneis 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 you finish your course.

Other treatments

If you are experiencing symptoms of chorea (uncontrollable physical jerking), you may be given a type of medication known as a neuroleptic. Neuroleptics work by blocking the nerve signals that are responsible for chorea.

A widely used neuroleptic in the treatment of chorea is called haloperidol, which is usually given in injection form until the symptoms are under control.

Common side effects of haloperidol include:

  • agitation,
  • problems sleeping,
  • headache,
  • dizziness,
  • changes in mood, such as feeling very low or depressed, and
  • problems with physical movement, such as trembling, having a shuffling unbalanced walk and unusually slow movements.

These side effects should pass once you stop taking haloperidol.

Symptoms

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

Rheumatic fever causes a wide range of symptoms, though you will not necessarily experience all of them. Symptoms include:

  • high temperature (fever) of or above 39°C (102°F),
  • a blotchy red skin rash,
  • headache,
  • weight loss,
  • nosebleeds,
  • fatigue,
  • excessive sweating,
  • chest pain,
  • shortness of breath,
  • abdominal pain,
  • vomiting,
  • joint pain and swelling (arthritis) – this usually begins in the knees and ankles and then spreads to other joints,
  • irritability,
  • shortened attention span,
  • changes in personality, such as moodiness, or unusual and inappropriate laughing and/or crying,
  • small nodules (bumps or lumps) develop under the skin, and
  • unusual and uncontrollable body movements, usually physical jerking of the ankles, knees, elbows and wrists (this is known as chorea).

The symptoms of arthritis usually pass within one to four weeks and the symptoms of fever within one to three weeks. Other symptoms may persist for around six weeks. If symptoms are particularly severe, they can last for several months.