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Rheumatic fever

Acute rheumatic fever

http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0004388/ US National Library of Medicine


Last reviewed: May 9, 2010.

Rheumatic fever is an inflammatory disease that may develop after an infection with Streptococcus bacteria (such as strep throat orscarlet fever). The disease can affect the heart, joints, skin, and brain.

Causes, incidence, and risk factors


Rheumatic fever is common worldwide and is responsible for many cases of damaged heart valves. It is not common in the United States, and usually occurs in isolated outbreaks. The latest outbreak was in the 1980s. Rheumatic fever mainly affects children ages 6 -15, and occurs approximately 20 days after strep throat or scarlet fever.

Symptoms
Abdominal pain Fever Heart (cardiac) problems, which may not have symptoms, or may result in shortness of breath and chest pain Joint pain, arthritis (mainly in the knees, elbows, ankles, and wrists) Joint swelling; redness or warmth Nosebleeds (epistaxis) Skin nodules Skin rash (erythema marginatum) Skin eruption on the trunk and upper part of the arms or legs Eruptions that look ring-shaped or snake-like

Sydenham chorea (emotional instability, muscle weakness and quick, uncoordinated jerky movements that mainly affect the face, feet, and hands)

Signs and tests


Because this disease has different forms, no one test can firmly diagnose it. Your doctor will perform a careful exam, which includes checking your heart sounds, skin, and joints. Tests may include: Blood test for recurrent strep infection (such as an ASO test) Complete blood count Electrocardiogram Sedimentation rate (ESR)

Several major and minor criteria have been developed to help standardize rheumatic fever diagnosis. Meeting these criteria, as well as having evidence of a recent streptococcal infection, can help confirm that you have rheumatic fever. The major criteria for diagnosis include: Arthritis in several joints (polyarthritis) Heart inflammation (carditis) Nodules under the skin (subcutaneous skin nodules) Rapid, jerky movements (chorea, Sydenham chorea) Skin rash (erythema marginatum)

The minor criteria include: Fever High ESR Joint pain Other laboratory findings

You'll likely be diagnosed with rheumatic fever if you meet two major criteria, or one major and two minor criteria, and have signs that you've had a previous strep infection.

Treatment
If you are diagnosed with acute rheumatic fever you will be treated with antibiotics. Anti-inflammatory medications such as aspirin or corticosteroids reduce inflammation to help manage acute rheumatic fever. You may have to take low doses of antibiotics (such as penicillin, sulfadiazine, or erythromycin) over the long term to prevent strep throat from returning.

Expectations (prognosis)
Rheumatic fever is likely to come back in people who don't take low-dose antibiotics continually, especially during the first 3 -5 years after the first episode of the disease. Heart complications may be severe, particularly if the heart valves are involved.

Complications
Arrhythmias Damage to heart valves (in particular, mitral stenosis and aortic stenosis) Endocarditis Heart failure Pericarditis Sydenham chorea

Calling your health care provider


Call your health care provider if you develop symptoms of rheumatic fever. Because several other conditions have similar symptoms, you will need careful medical evaluation. If you have symptoms of strep throat, tell your health care provider. You will need to be evaluated and treated if you do have strep throat, to decrease your risk of developing rheumatic fever.

Prevention
The most important way to prevent rheumatic fever is by getting quick treatment for strep throat and scarlet fever.

References
1. Bisno AL, Stevens DL. Streptococcus pyogenes. In: Mandell GL, Bennett JE, Dolin R, eds. Principles and Practice of Infectious Diseases. 7th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2009:chap 198.
Review Date: 5/9/2010. Reviewed by: Linda Vorvick, MD, Seattle Site Coordinator, Lecturer, Pahtophysiology, MEDEX Northwest Division of Physician Assistant Studies, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

Rheumatic fever
Definition
By Mayo Clinic staff Rheumatic fever is an inflammatory disease that can develop as a complication of inadequately treated strep throat. Strep throat is caused by infection with group A streptococcus bacteria. Rheumatic fever is most common in 5- to 15-year-old children, though it can develop in younger children and adults. Although it's very rare in United States and other developed countries, rheumatic fever remains common in many developing nations. Rheumatic fever can cause permanent damage to the heart, including damaged heart valves and heart failure. Treatments can reduce tissue damage from inflammation, lessen pain and other symptoms, and prevent the recurrence of rheumatic fever.

Symptoms
By Mayo Clinic staff Rheumatic fever symptoms may vary. Some people may have several symptoms, while others experience only a few. The symptoms may also change during the course of the disease. The onset of rheumatic fever usually occurs about two to four weeks after a strep throat infection. Rheumatic fever signs and symptoms which result from inflammation in the heart, joints, skin or central nervous system may include: Fever Painful and tender joints most often the ankles, knees, elbows or wrists; less often the shoulders, hips, hands and feet Pain in one joint that migrates to another joint Red, hot or swollen joints Small, painless nodules beneath the skin Chest pain Sensation of rapid, fluttering or pounding heartbeats (palpitations)

Fatigue Shortness of breath Flat or slightly raised, painless rash with a ragged edge (erythema marginatum) Jerky, uncontrollable body movements (Sydenham chorea or St. Vitus' dance) most often in the hands, feet and face

Outbursts of unusual behavior, such as crying or inappropriate laughing, that accompanies Sydenham chorea When to see a doctor Your child should see a doctor if he or she has signs or symptoms of strep throat. Proper treatment of strep can prevent rheumatic fever. Call your doctor if your child has any of the following signs or symptoms:

A sore throat without cold symptoms, such as a runny nose A sore throat accompanied by tender, swollen lymph glands (nodes) Rash Difficulty swallowing anything, including saliva Thick or bloody discharge from the nose, which is more likely in children under 3 years of age Call your doctor about a fever in the following situations:

Newborns up to 6 weeks with a fever of 100 F (37.8 C) Children ages 6 weeks to 2 years with a temperature of 102 F (38.9 C) or higher Children age 2 or older with a fever of 103 F (39.4 C) or higher Any fever that lasts more than three days Also, see your doctor if your child shows any other signs or symptoms of rheumatic fever.

Causes
By Mayo Clinic staff Rheumatic fever can occur after an infection of the throat with a bacterium called Streptococcus pyogenes, or group A streptococcus. Group A streptococcus infections of the throat cause strep throat or,

less commonly, scarlet fever. Group A streptococcus infections of the skin or other parts of the body rarely trigger rheumatic fever. The exact link between strep infection and rheumatic fever isn't clear, but it appears that the bacterium "plays tricks" on the immune system. The strep bacterium contains a protein similar to one found in certain tissues of the body. Therefore, immune system cells that would normally target the bacterium may treat the body's own tissues as if they were infectious agents particularly tissues of the heart, joints, skin and central nervous system. This immune system reaction results in inflammation. If your child receives prompt and complete treatment with an antibiotic to eliminate strep bacteria in other words, taking all doses of the medication as prescribed there's little to no chance of developing rheumatic fever. If your child has one or more episodes of strep throat or scarlet fever that aren't treated or not treated completely, he or she may but won't necessarily develop rheumatic fever.

Risk factors
By Mayo Clinic staff Factors that may increase the risk of rheumatic fever include: Family history. Some people may carry a gene or genes that make them more likely to develop rheumatic fever. Type of strep bacteria. Certain strains of strep bacteria are more likely to contribute to rheumatic fever than are other strains. Environmental factors. A greater risk of rheumatic fever is associated with overcrowding, poor sanitation, and other conditions that may easily result in the rapid transmission or multiple exposures to strep bacteria.

Complications
By Mayo Clinic staff Inflammation caused by rheumatic fever may last for a few weeks to several months. In some cases, the inflammation may cause long-term complications. Rheumatic heart disease is permanent damage to the heart caused by the inflammation of rheumatic fever. Problems are most common with the valve between the two left chambers of the heart (mitral valve), but the other valves may be affected. The damage may result in one of the following conditions: Valve stenosis. This condition is a narrowing of the valve, which results in decreased blood flow.

Valve regurgitation. This condition is a leak in the valve, which allows blood to flow in the wrong direction.

Damage to heart muscle. The inflammation associated with rheumatic fever can weaken the heart muscle, resulting in poor pumping function. Damage to the mitral valve, other heart valves or other heart tissues can cause problems with the heart later in life. Resulting conditions may include:

Atrial fibrillation, an irregular and chaotic beating of the upper chambers of the heart (atria) Heart failure, an inability of the heart to pump enough blood to the body

Tests and diagnosis


By Mayo Clinic staff Your doctor makes a diagnosis of rheumatic fever based on: Signs and symptoms you report Evidence of inflammation Evidence of recent group A streptococcal infection Physical exam Your child's doctor will conduct a thorough physical examination that may include: Checking the joints for signs of inflammation Examining the skin for nodules under the skin or a rash Listening to the heart for abnormal rhythms, murmurs or muffled sounds that may indicate inflammation of the heart Conducting a series of simple movement tests to detect indirect evidence of inflammation of the central nervous system Tests for strep infection If your child was already diagnosed with a strep infection, your doctor may not order any additional tests for the bacterium. If your doctor orders a test, it will most likely be a blood test that can detect antibodies to strep bacteria circulating in the blood. The actual bacteria may no longer be detected in your child's throat tissues or blood.

Electrocardiogram (ECG) An electrocardiogram also called an ECG or EKG records electrical signals as they travel through your child's heart. Your doctor can look for patterns among these signals that indicate inflammation of the heart or poor heart function. Echocardiography An echocardiogram uses sound waves to produce live-action images of the heart. This common test may enable your doctor to detect altered structures within the heart. Damage to heart valves isn't likely to occur early in the disease, but an echocardiogram can show such problems.

Treatments and drugs


By Mayo Clinic staff The goals of treatment for rheumatic fever are to destroy any remaining group A streptococcal bacteria, relieve symptoms, control inflammation and prevent recurring episodes of rheumatic fever. Treatments used for rheumatic fever include: Antibiotics. Your child's doctor will prescribe penicillin or another antibiotic to eliminate any remaining strep bacteria that may exist in your child's body. After your child has completed the full antibiotic treatment, your doctor will begin another course of antibiotics to prevent recurrence of rheumatic fever. This preventive treatment usually continues until your child is at least 20 years old. If an older teenager has had rheumatic fever, he or she may continue taking the antibiotics past age 20 to complete a minimum five-year course of preventive treatment. People who experienced inflammation of the heart when they had rheumatic fever may be advised to take the preventive antibiotic treatment much longer or even for life. Anti-inflammatory treatment. Your doctor will prescribe a pain reliever, such as aspirin or naproxen (Anaprox, Naprosyn, others), to reduce inflammation, fever and pain. If symptoms are severe or your child isn't responding to the anti-inflammatory drugs, your doctor may prescribe a corticosteroid, such as prednisone. Anticonvulsant medications. If the involuntary movements of Sydenham chorea are severe, your doctor may prescribe an anticonvulsant, such as valproic acid (Depakene, Stavzor) or carbamazepine (Carbatrol, Equetro, others).

Long term care Discuss with your doctor what type of follow-up and long term care your child will need. Heart damage from rheumatic fever may not show up until many years after the acute illness. Your child should be informed that he or she had rheumatic fever, and when an adult should discuss this with his or her doctor.

Prevention
By Mayo Clinic staff The only known way to prevent rheumatic fever is to treat strep throat infections or scarlet fever promptly with a full course of appropriate antibiotics.

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