Arthritis and Rheumatology Conditions and Diagnoses

Rheumatic Fever

What is rheumatic fever?

Rheumatic fever is a systemic illness that can affect the joints, skin, heart, blood vessels, and brain. It is a systemic immune disease that may develop after an infection with streptococcus bacteria, such as strep throat and scarlet fever.

What causes rheumatic fever?

Rheumatic fever is a delayed reaction by the immune system to proteins in the streptococcus bacteria. It can be prevented in the vast majority of cases with prompt diagnosis of the infection with the strep bacteria and effective treatment with antibiotics. Rheumatic fever is uncommon in the US, except in children who have had strep infections that were untreated or inadequately treated. However, in many cases of rheumatic fever there were no symptoms suggesting strep throat or the symptoms were very mild, similar to a cold, and no sore throat was reported to occur.

What are the symptoms of rheumatic fever?

The symptoms of rheumatic fever usually start about one to five weeks after your child has been infected with streptococcus bacteria. The following are the most common symptoms of rheumatic fever. However, each child may experience symptoms differently. Each patient will demonstrate some, but not all of these symptoms. They may include:

Joint inflammation - including swelling, tenderness, and redness in one or many joints. The joints affected are usually the larger joints in the knees or ankles. The inflammation "moves" from one joint to another over several days. The joints are very tender and very painful with movement.
Small nodules or hard, round bumps under the skin
A change in your child's neuromuscular movements so that they have involuntary movements while they are awake (this is usually noted by a change in your child's handwriting and may also include jerky movements)
Rash - a pink rash with irregular shaped border that is usually seen on the trunk of the body or arms and legs
Fever
Weight loss
Fatigue
Stomach pains

Who is at risk for developing rheumatic fever?

Children ages 5 to 15, particularly if they experience frequent strep throat infections, are most at risk for developing rheumatic fever. Rheumatic fever is also more common in children who have a family history of rheumatic fever. There is an increased frequency of rheumatic fever in the winter and spring, as strep throats occur more frequently during these seasons. Strep infections are contagious, but rheumatic fever is not.

How is rheumatic fever diagnosed?

In addition to a complete medical history and physical examination, rheumatic fever is diagnosed by your child's physician based on the presence of criteria found in the revised modified Jones criteria diagnostic tool (standard guidelines for diagnosis of rheumatic fever). The Jones criteria have been used for many years as the standard for making the diagnosis of rheumatic fever and use major and minor criteria.

Major criteria include:

Carditis (inflammation of the heart)
Polyarthritis (inflammation of more than one joint)
Chorea (unusual jerky movements, most often involving the face and hands)
Subcutaneous nodules (small, painless bumps under the skin, often over bony areas)
Rash (a red, irregular rash on the trunk)

Minor criteria include:

Fever
Arthralgia (pain in one or more joints)
Previous episode of rheumatic fever related carditis (inflammation of the heart)
Changes in the electrocardiogram (EKG) pattern
Abnormal sedimentation rate or C-reactive protein (laboratory tests performed on blood)

The diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of a streptococcal infection. Evidence of a streptococcal infection can be either a throat culture or blood tests showing the presence of antibodies to the streptococcal bacteria.

Blood work is done to assist in making a diagnosis. Your child's physician may also order an electrocardiogram, or EKG (a test that records the electrical activity of the heart). The EKG is very safe and may show abnormal heart rhythms, inflammation of the heart, or damage of the heart muscle) as part of the diagnostic process for rheumatic fever. A throat culture may also be done to determine if the child tests positive for streptococcus bacteria, although during the initial phase of rheumatic fever, the throat culture is often negative.

Treatment for Rheumatic Fever
Specific treatment for rheumatic fever will be determined by your child's physician based on:

Type and severity of the manifestations of your child’s rheumatic fever episode
Your child's overall health and medical history
Extent of the reaction
Your child's tolerance for specific medications, procedures, or therapies
Expectations for the course of the reaction
Your opinion or preference

Children with rheumatic fever are often treated in the hospital, depending upon the severity of the disease.

Treatment for rheumatic fever, in most cases, combines the following three approaches:

Treatment for streptococcus infection
The immediate goal is to treat the infection with antibiotics. This is done even if the throat culture is negative. Following the initial treatment for strep infection, your child will receive ongoing therapy to prevent streptococcal infections in the future to decrease the risk for additional episodes of rheumatic fever.
Anti-inflammatory medications
Based on the severity of your child's condition, your child's physician will prescribe medications to decrease the inflammation in the heart muscle or joints.
Bed rest
The length of bed rest will be determined by your child's physician, based on the severity of your child's disease and the involvement of the heart and joints. Bed rest may range from two to twelve weeks.

Are there any complications from having rheumatic fever?

Depending on the severity of the initial attack of the disease on the heart, some children may develop heart disease. Physical activity and sports may be restricted in your child, based on your child's physician's findings.
Also, if your child had heart involvement during the initial course of rheumatic fever, he/she will need to receive antibiotics before having dental work done. This helps decrease the chance of infection migrating to the heart during the dental procedure. Consult your child's physician for more information. The joint inflammation in rheumatic fever responds very quickly and completely to treatment and no joint damage occurs.

Can my child develop rheumatic fever again?

Yes, although the chances are reduced because of the use of antibiotics after the initial disease process. The greatest chance of recurrence is during the first three years. The chance of developing the disease again decreases with age and time since the first attack. If there is another episode of rheumatic fever, there is greater risk for worsening of the heart involvement or if there was not heart involvement in the first episode it may develop in later episodes.

After having rheumatic fever, your child will need medications on a regular ongoing basis to help decrease the chance of developing rheumatic fever again. Usually by the time your child is 18 and if your child's physician feels he / she is at low risk for developing heart disease, the antibiotic therapy may be stopped. Close follow-up with your child's physician is needed.