Rheumatic fever is an inflammatory disease that is a complication of untreated strep throat. It affects the body's connective tissues — especially those of the heart, joints, brain, or skin. Rheumatic fever damages connective tissue by causing it to swell, but its greatest danger lies in the damage it does to the connective tissue in the heart—the heart valves. Before antibiotic medicines became widely used, it was the most common cause of heart valve disease. More than half of the time, rheumatic fever scars the heart valves, and this scarring narrows the valve to the point that it cannot open properly or close completely. In turn, the heart must work harder to pump blood to the rest of the body. This valve damage can lead to a condition called rheumatic heart disease, which, in time, can lead to congestive heart failure. The effects of rheumatic heart disease last for life.
What Causes Rheumatic Fever?
Updated December 2009
Rheumatic fever is not an infection itself. Rather, it is the result of an untreated strep infection. The immune system attempts to fight the strep infection with antibodies, but the antibodies become overactive and attack the body’s own connective tissue. This causes the heart valves to swell and the heart valve leaflets to become scarred. After the damage is done, the valve cannot open or close properly.
Who is at Risk for Rheumatic Fever?
Less than 3% of people with untreated strep throat get rheumatic fever. Anyone can develop acute rheumatic fever, but children 5 to 15 years old are especially at risk. Women are also more likely than men to develop rheumatic fever. In 2004, rheumatic fever and rheumatic heart disease killed 3,248 people in the United States; this is dramatically decreased from 1950, when 15,000 deaths were recorded. A weakened immune system may be a factor in developing rheumatic fever. And, although antibiotic medicines have reduced the number of cases of rheumatic fever in developed countries, thousands of cases are still reported. It is a worldwide problem.
The symptoms of rheumatic fever usually begin 1 to 6 weeks after a strep infection. They vary greatly but include:
• Joint pain in the wrists, elbows, knees, or ankles
• Joint swelling, redness, or warmth
• Skin problems:
o Small bumps called nodules under the skin over the elbows or knees
o A raised, red rash on the chest, back, or stomach
o Skin eruption on the trunk and upper part of the arms or legs
o Eruptions that look ring-shaped or snake-like
• Abdominal pain or loss of appetite
• Weakness, shortness of breath, or fatigue
• Sydenham's chorea (emotional instability, muscle weakness, and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands)
• Heart problems, with or without symptoms, including shortness of breath and chest pain
The heart valve damage may not be immediately noticeable. It often takes time to develop and present itself. If severe enough, the heart valve disease may lead to further complications, such as congestive heart failure or bacterial endocarditis.
A throat culture to test for strep infection is performed. A stethoscope is used to listen for heart murmurs. A physical examination is performed to look for nodules on joints and other sign of rheumatic fever. Blood tests, chest x-rays, or an electrocardiogram (ECG) may be needed for a more definite diagnosis.
Rheumatic fever can be prevented by seeking immediate treatment for sore throat pain. If rheumatic fever does develop and leads to rheumatic heart disease or heart valve damage, antibiotic medicine may be prescribed continuously for many years (or for the rest of the patient’s life). Anti-inflammatory medicines or aspirin to reduce tissue swelling may also be prescribed. A diuretic medicine may help rid the body of excess water and salt. The length of treatment is determined by the patient’s age and the severity of the disease and the symptoms. In some patients, the damage caused by rheumatic fever is severe enough to warrant heart valve repair or replacement.
The AHA guidelines no longer recommend antibiotic prophylaxis prior to dental or surgical procedures for patients with rheumatic heart disease unless they also have a prosthetic cardiac valve, previous endocarditis, a specific form of congenital heart disease, or any condition that puts them at an especially high risk of developing bacterial endocarditis.
Texas Heart Institute www.texasheartinstitute.com/HIC/Topics/Cond/rheufev.cfm
American Heart Association www.americanheart.org/presenter.jhtml?identifier=4709
Medline Plus www.nlm.nih.gov/medlineplus/ency/article/003940.htm