Rheumatic fever

Other Names:

• Acute Rheumatic Fever; Inflammatory Rheumatism

Rheumatic fever

• Rheumatic fever is an inflammatory condition that may develop after infection with group A Streptococcus bacteria, such as strep throat or scarlet fever.
• It is primarily diagnosed in children between the ages of 6 and 16 and can affect the heart, joints, nervous system and/or skin.

Cause

• Rheumatic fever may develop after strep throat or scarlet fever infections that are not treated properly. Bacteria called group A Streptococcus or group A strep cause strep throat and scarlet fever.
• It usually takes about 1 to 5 weeks after strep throat or scarlet fever for rheumatic fever to develop.
• Rheumatic fever is thought to be caused by a response of the body’s defense system — the immune system.
• The immune system responds to the earlier strep throat or scarlet fever infection and causes a generalized inflammatory response.
• The exact underlying cause of the condition is not well understood and it is unclear why some people with strep infections go on to develop rheumatic fever, while others do not.
• However, some scientists suspect that an exaggerated immune response in genetically susceptible people may play a role in the development of the condition.

Transmission

• People cannot catch rheumatic fever from someone else because it is an immune response and not an infection.
• However, people with strep throat or scarlet fever can spread group A strep to others, primarily through respiratory droplets.

Inheritance

• Rheumatic fever is likely inherited in a multifactorial manner, which means it is caused by multiple genes interacting with each other and with environmental factors.
• The condition is thought to occur in genetically susceptible children who are infected with group A Streptococcus bacteria and live in poor social conditions.
• Some studies suggest that differences in the expression of various genes involved in the immune response may contribute to rheumatic fever susceptibility.

Riskfactors

Although anyone can get rheumatic fever, it is more common in school-age children (5 through 15 years old). Rheumatic fever is very rare in children younger than three years old and adults. Infectious illnesses, including group A strep, tend to spread wherever large groups of people gather together. Crowded conditions can increase the risk of getting strep throat or scarlet fever, and thus rheumatic fever. These settings include:

• Schools
• Daycare centers
• Military training facilities
• Someone who had rheumatic fever in the past is more likely to get rheumatic fever again if they get strep throat or scarlet fever again.

Signs and Symptoms

• Rheumatic fever is primarily diagnosed in children between the ages of 6 and 16 and can affect many different systems of the body, including the heart, joints, nervous system and/or skin.
• The condition usually develops approximately 14-28 days after infection with group A Streptococcus bacteria, such as strep throat or scarlet fever. Early signs and symptoms may include sore throat; swollen red tonsils; fever; headache; and/or muscle aches.

Affected people may also experience:

• Fever
• Painful, tender joints (arthritis), most commonly in the knees, ankles, elbows, and wrists
• Symptoms of congestive heart failure, including chest pain, shortness of breath, fast heartbeat
• Fatigue
• Jerky, uncontrollable body movements (called “chorea”)
• Painless lumps (nodules) under the skin near joints (this is a rare symptom)
• Rash that appears as pink rings with a clear center (this is a rare symptom)

In addition, someone with rheumatic fever can have:

• A new heart murmur
• An enlarged heart
• Fluid around the heart

Diagnosis

There is no single test used to diagnose rheumatic fever. Instead, doctors can look for signs of illness, check the patient’s medical history, and use many tests, including:

• A throat swab to look for a group A strep infection
• A blood test to look for antibodies that would show if the patient recently had a group A strep infection
• A test of how well the heart is working (electrocardiogram or EKG)
• A test that creates a movie of the heart muscle working (echocardiography or echo)

• The diagnosis can also be supported by blood tests that confirm the presence of certain proteins that increase in response to inflammation (called acute-phase reactants) and tend to be elevated in rheumatic fever.
• Additional tests may be recommended to rule out other conditions that cause similar features.

Treatment

• Treatment of rheumatic fever usually consists of antibiotics to treat the underlying group A Streptococcus bacterial infection and anti-inflammatory medications such as aspirin or corticosteroids.
• Because people with a history of rheumatic fever have a high risk of developing recurrent episodes of the condition, low dose antibiotics are often continued over a long period of time to prevent recurrence.

Prognosis

• The long-term outlook (prognosis) for people with rheumatic fever depends on the severity of the heart involvement at the initial diagnosis.
• Severe carditis (inflammation of the heart) is associated with a poor prognosis and generally leads to rheumatic heart disease.
• People with a history of rheumatic fever have a high risk of developing recurrent episodes of the condition, which can cause progressive (worsening over time) heart damage.
• Prophylactic low dose antibiotics can significantly improve prognosis in many cases by preventing these recurrences.

Prevention

• Having a group A strep infection does not protect someone from getting infected again in the future.
• People can also get rheumatic fever more than once. However, there are things people can do to protect themselves and others.
• Good Hygiene Helps Prevent Group A Strep Infections
• The best way to keep from getting or spreading group A strep infections such as strep throat or scarlet fever is to wash your hands often, especially after coughing or sneezing and before preparing foods or eating.
• Antibiotics Are Key to Treatment and Prevention.

The main ways to prevent rheumatic fever are to

• Treat group A strep infections like strep throat and scarlet fever with antibiotics
• Prevent group A strep infections in the first place
• Use preventive antibiotics for people who had rheumatic fever in the past
• Preventive antibiotics help protect people who had rheumatic fever from getting it again. Doctors also call this prophylaxis (pro-fuh-LAK-sis) or “secondary prevention.” People may need antibiotic prophylaxis over a period of many years (often until 21 years old). Prophylaxis can include daily antibiotics by mouth or a shot into the muscle every few weeks.

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