Rheumatic fever: Causes, symptoms, and treatment

Rheumatic fever is an inflammatory reaction that can develop as a complication of a Group A streptococcal infection, such as strep throat or scarlet fever. It occurs when the infection has been untreated or undertreated.

Not everyone who has a streptococcal infection will develop rheumatic fever (RF), but if RF does develop, symptoms normally appear 2 to 4 weeks after infection.

It most commonly affects boys and girls aged 5 to 15 years, but it can occur in adults and younger children. Neurologic complications seem to be more common in females.

RF can have long-term complications, the most common being rheumatic heart disease (RHD) which develops in 30 to 45 percent of those with RF. Worldwide, RHD is responsible for 230,000 – 500,000 deaths per year.

Before the widespread introduction of antibiotics, RF was a leading cause of acquired heart disease in developed nations, but it is now relatively rare in these countries. Thanks to routine treatment of Strep throat, RF now only occurs in about 0.04-0.06 cases per every 1,000 children in mainland U.S.

Symptoms

[boy with fever]
Rheumatic fever is a complication of some types of streptococcal infection.

RF is caused by a reaction to the bacteria that cause strep throat, so that diagnosis and treatment of this condition can prevent it from developing into RF.

Symptoms of strep throat include:

sore throat

headache

swollen, tender lymph nodes

trouble swallowing

nausea and vomiting

red skin rash

high temperature

swollen tonsils

abdominal pain

Signs and symptoms generally develop 2 to 4 weeks after a streptococcal infection.

Some individuals will experience just one or two of the following symptoms, but others may experience most of them:

fatigue

rapid heart rate

decreased ability to exercise

joint pain and swelling

fever

splotchy rash

uncontrollable twitching and movements

Arthritis, or pain and swelling in the joints, affects 75 percent of patients. It normally starts in the larger joints, such as the knees, ankles, wrists, and elbows, before moving to other joints. This inflammation normally resolves within 4-6 weeks, without causing permanent damage.

Inflammation of the heart can lead to chest pain, palpitations, a sensation that the heart is fluttering or pounding hard, panting, and shortness of breath, and fatigue.

On average, around 50 percent of patients develop carditis or valvulitis, a potentially fatal inflammation of the heart that can have serious, long-term effects. Younger children are more susceptible.

Inflammation of the nerves can lead to symptoms of Sydenham’s chorea, including:

chorea, the uncontrollable jerking of knees, elbows, wrists, and ankles

inappropriate crying or laughing

irritability and moodiness

difficulty controlling fine hand movements

problems with balance

Symptoms usually pass within a few months but can last up to 2 years. They are not normally permanent.

Other symptoms include a red, blotchy, skin rash, which appears in 1 in 10 cases. Less common are nosebleeds, abdominal pain, bumps and lumps, or nodules, under the skin, and a high fever over 102 degrees Fahrenheit.

The inflammation may also lead to headache, sweating, vomiting, and weight loss.

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Treatment

Treatment aims to destroy the bacteria, relieve symptoms, control inflammation, and prevent recurrences of RF.

Antibiotics, such as penicillin, may be given to destroy any remaining strep bacteria in the body. Further antibiotics may be prescribed, to prevent recurrence. This may continue for 5-10 years depending on the age of the person and whether or not the heart is affected.

Long-term, and even lifelong, preventive antibiotics may be necessary to prevent recurring inflammation of the heart.

It is important to remove all traces of streptococcal bacteria, as any remaining bacteria can lead to repeated occurrences of RF and a significantly higher risk of heart damage, which can become permanent.

Anti-inflammatory drugs: Naproxen, for example, may help to reduce pain, inflammation, and fever.

Corticosteroids: Prednisone may be given if the patient does not respond to first-line anti-inflammatory medications, or if there is inflammation of the heart.

Aspirin: This is not usually recommended for children aged under 16 years because of the risk of developing Reye’s syndrome, which can cause liver and brain damage, and even death, but an exception is usually made in cases of RA because the benefits are greater than the risks.

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