Rheumatic Fever

Early strep throat treatment helps prevent more serious related conditions like rheumatic fever.

• Rheumatic fever is a complication of a streptococcal pharyngitis infection (strep throat) that can cause damage to the heart, joints, brain, and skin.
• The most serious complication of rheumatic fever is rheumatic heart disease (RHD). RHD is the most common cause of heart problems in children worldwide and can lead to damage to the heart valves and chronic heart failure.
• Rheumatic fever is preventable by treating strep throat with antibiotics, usually penicillin. If a patient is allergic to penicillin, other antibiotics such as erythromycin (Eryc, Ery-Tab, E.E.S, Eryped, PCE) or clindamycin (Cleocin) can be used.
• The use of antibiotics and improved sanitation has dramatically reduced rheumatic fever in developed countries.

• Rheumatic fever is a complication of strep throat caused by infections with group A streptococcal bacteria.
• After strep throat, some individuals can develop a second illness one to five weeks later with fever, joint pains, rash, and sometimes brain and heart problems.

While it is not completely clear, rheumatic fever seems to be caused by a process called "molecular mimicry."

• During infections with bacteria, the immune system fights the infection by producing antibodies to proteins on the surface of the bacteria.
• During infection with certain types (or strains) of group A streptococcal bacteria, the proteins on the bacteria appear similar to proteins in the human body.
• Because of this similarity, the immune system begins to attack human cells with similar proteins, such as heart muscle, joints, skin, and sometimes brain tissue.

The primary risk for rheumatic fever is a recent bout of strep throat. Other infections with group A streptococci may also lead to rheumatic fever; one such condition is called pyoderma (a skin infection). Age is also a risk factor. Rheumatic fever occurs most commonly before the age of 35 and is most frequent in children.

• The main symptom of rheumatic fever is a fever one to five weeks after strep throat. The fever is usually under 102 F and improves with acetaminophen (Tylenol) or ibuprofen (Advil).
• Acute rheumatic fever is often accompanied by joint swelling and pain (arthritis). Arthritis occurs in 75% of first attacks of rheumatic fever. Arthritis due to rheumatic fever commonly involves painful joint swelling that can move from joint to joint. The arthritis often involves the large joints such as knees, shoulders, and hips.
• Heart problems (carditis), occur in about half the cases of rheumatic fever. The most common and important heart problem due to rheumatic fever is inflammation and eventually destruction of heart valves. Destruction of the heart valves can lead to heart failure.
• Sydenham's chorea is another sign of rheumatic fever. Chorea is sudden involuntary movements of muscles due to irritation of specific areas of the brain. In about 10% of people with rheumatic fever, sudden movements of the face, arms, and hands occur up to six months after the start of fever and can last one to two months.
• Skin problems can occur as a result of rheumatic fever in about 2% of people. The signs of skin involvement are erythema marginatum, which is a pink rash that looks serpentine and surrounds areas of normal looking skin. Lumps over bones such as the knees and elbows can also occur. The lumps, or subcutaneous nodules, are round and painless. The nodules usually appear several weeks after the beginning of fever.

Rheumatic fever is diagnosed by applying a set of guidelines called the Jones criteria to a patient who has had a recent streptococcal infection.

To diagnose rheumatic fever, a patient must have had a recent strep infection diagnosed by throat culture, rapid strep test, or have antibodies in the blood to strep (known as a positive ASO or antistreptolysin O titer).

In addition to a recent strep infection, a patient must either have two "major" criteria, or one major criteria and two "minor" criteria (signs /symptoms) from the following chart.

Treating rheumatic fever involves several important steps.

• First, if the patient has an active strep infection, they are treated with penicillin. If they are allergic to penicillin, other antibiotics such as erythromycin may be used.
• Once the strep infection is treated, the next step is to determine if the heart is being affected by rheumatic fever. To do this, additional testing such as a heart ultrasound (echocardiogram) may be done.
• At the same time, treatment is started to stop the attack on the organs affected by rheumatic fever. This is done with anti-inflammatory medications. Aspirin is the mainstay of rheumatic fever therapy, but alternative drugs including NSAIDs (such as ibuprophen) or with steroids (such as prednisone) are often used.
• If the heart is severely involved, treatment for heart failure may be necessary.

Rheumatic fever will go resolve spontaneously within 12 weeks even if is not treated.

• With treatment, it can resolve within two weeks.

The main complication of rheumatic fever is rheumatic heart disease (RHD).

• RHD can lead to destruction of the heart valves.
• If this occurs, repair or replacement of the heart valves with either a mechanical heart valve or an organic heart valve (pig valve) may be necessary to prevent heart failure.

The ultimate prognosis, however, is determined by the level of heart involvement with rheumatic fever.

• If the heart is severely affected, the patient may go on to develop rheumatic heart disease. If not treated, rheumatic heart disease can cause scarring of the heart valves such as mitral stenosis or aortic stenosis.
• If not treated, destruction and scarring of the valves can lead to heart failure.

Unfortunately, if a person has had one bout of rheumatic fever, he or she is at higher risk for future bouts of rheumatic fever.

• The risk seems to be highest in the first 10 years after the first bout of rheumatic fever.
• Because of this risk, most patients who have had one bout episode of rheumatic fever will be placed on long-term antibiotics to prevent another strep infection.
• This is usually done with either penicillin by injection every three to four weeks or by taking daily penicillin by mouth.
• If the patient is allergic to penicillin, other antibiotics such as erythromycin or clindamycin can be used.

Long term, patients who have had one bout of rheumatic fever are at higher risk to develop rheumatic fever with future strep infections.

In addition to preventing future infections, most patients who have had rheumatic fever are carefully monitored with heart ultrasounds (echocardiograms) every one to two years. If the patient had serious heart involvement with the first bout of rheumatic fever, they may be monitored even more closely with echocardiograms as often as every three to six months to watch for heart problems.

If a person with rheumatic fever has any heart involvement at all, they will be on lifelong prophylaxis with antibiotics for dental work and should have yearly dental exams.

• While no vaccine currently exists to prevent rheumatic fever, research is ongoing to try to develop a safe and effective vaccine against strep bacteria.
• In the meantime, the most effective way to prevent rheumatic fever is to diagnose and treat strep throat with antibiotics. It is important to remember that not all sore throats are due to strep. In fact, most sore throats are due to viruses and do not need treatment with antibiotics. Classic strep throat has high fever with a very sore throat and does not usually have nasal congestion or cough. The diagnosis can be made by your doctor with decision-making tools or with testing by throat culture or rapid strep tests.