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Rheumatic fever is an inflammatory disease that is rare in the United States but common in some other parts of the world. It primarily affects children between the ages of 6 and 16, and develops after an infection with streptococcal bacteria, such as strep throat or scarlet fever. About 5% of those with untreated strep infection will develop rheumatic fever.
Rheumatic fever may affect the heart, joints, nervous system and/or skin. In more than half of all cases, it leads to serious inflammatory disease of the valves of the heart. Joint disease is the second most common consequence of rheumatic fever.
The streptococcal infection that precedes rheumatic fever may or may not be noticeable as a sore throat. After a latent period of two or three weeks, the patient may develop symptoms of rheumatic fever. The most common symptoms are: sore throat; swollen, red tonsils; fever; headache; and joint and muscle aches, especially in the knees.
The most serious problem that can be caused by rheumatic fever is rheumatic heart disease. The membranes lining the heart chambers may be inflamed (endocarditis), the muscle walls of the heart may be inflamed (myocarditis), the membrane surrounding the heart may be inflamed (pericarditis), or any combination of these symptoms may occur. A heart murmur not previously present, enlargement of the heart (cardiomegaly), congestive heart failure, and pericardial friction rubs or leakage of blood from vessels into heart tissue may be signs of rheumatic carditis. Inflammation and subsequent scarring of heart valves occurs in patients with this disorder, and can lead to heart function abnormalities.
If the disorder affects the nervous system the patient may present with abrupt, non-repetitive limb movements and grimaces (Sydenham's chorea) that typically become apparent within a couple of months after the initial streptococcal infection. Such symptoms may disappear after a few weeks or months.
If the disorder affects connective tissue or the autoimmune system, painless, firm, round lumps underneath the skin (subcutaneous nodules) may develop over bones and near joints. The nodules rarely last for more than a month. However, the associated arthritis may last for some time.
Heart inflammation (carditis), chorea and arthritis are complications of rheumatic fever that can occur singly or in combination. Subcutaneous nodules and a typical rash (erythema marginatum) are rarely seen without carditis. Moderate fever, a general feeling of discomfort (malaise), and fatigue usually occur, especially when carditis is present.
Although rheumatic fever is clearly linked to Group A streptococcal infections (strep throat), the exact mechanism causing the disorder is not well understood. Strep throat is highly contagious, whereas rheumatic fever is not contagious. People who have had rheumatic fever tend to develop flare-ups with repeated strep infections.
Rheumatic fever usually affects children between the ages of 6 and 16, but may occur among young adults as well. Although outbreaks have steadily declined since the end of World War II in the United States as a result of the use of antibiotics such as penicillin and its derivatives, several outbreaks linked to a particularly virulent strain of streptococcal infection have occurred.
However, throughout this period, this disorder has remained a constant ailment in India, the Middle East and some countries in Africa.
Symptoms of the following disorder can be similar to those of rheumatic fever. Comparisons may be useful for a differential diagnosis:
Juvenile rheumatoid arthritis, also known as Still's disease or chronic polyarthritis, is characterized by progressive pain and tenderness in one or more joints. This disorder, which tends to affect girls more than boys, may begin abruptly with high fever, joint pain, and a variety of skin rashes. Normal growth may be diminished and the spleen and/or liver may become enlarged. The exact cause is not known. Some forms of arthritis are believed to be autoimmune disorders (the body's natural defenses against invading organisms suddenly begin to attack healthy tissue).
The diagnosis of rheumatic fever is made on the basis of a physical examination that finds either the presence of at least two major diagnostic criteria, or the presence of one major and at least two minor diagnostic criteria.
The major diagnostic criteria are:
Heart inflammation detected by shortness of breath and weakness, or by ECG or chest X-ray
Arthritic pain in the major joints, and movement of that pain from one joint to another
Involuntary, transient, jerky movements (chorea)
Broad, pink or faint-red, non-itching patches on the skin
The minor diagnostic criteria are:
Joint pain without inflammation
Prior RF or evidence of rheumatic heart disease
Positive blood test for inflammation
New heart murmors
Treatment is designed to reduce inflammation with anti-inflammatory medications such as aspirin or corticosteroids. People with positive cultures for strep throat should also be treated with antibiotics.
In addition, low-dose antibiotics, such as penicillin, sulfadiazine, or erythromycin, are taken over a period of time to prevent recurrence.
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Beers MH, Berkow R, eds. The Merck Manual, 17th ed. Whitehouse Station, NJ: Merck Research Laboratories; 1999:2397-402.
Berkow R, ed. The Merck Manual-Home Edition.2nd ed. Whitehouse Station, NJ: Merck Research Laboratories; 2003:1565-66.
Frank MM, Austen KF, Claman HN, et al., eds. Samter's Immunological Diseases. 5th ed. Little Brown and Company, Boston, MA; 1995:823-30.
Kelley WN, Harris ED, Ruddy S, et al. Textbook of Rheumatology. 4th ed. W. B. Saunders Company. Philadelphia, PA; 1993:1209-21.
Mackie SL, Keat A. Poststreptococcal reactive arthritis: what is it and how do we know? Rheumatology (Oxford). 2004;43:949-54.
Boudoulas H. Etiology of valvular heart disease. Expert Rev Cardiovasc Ther. 2003;1:523-32.
Tani LY, Veasy LG, Minich LL, et al. Rheumatic fever in children younger than 5 years: is the presentation different? Pediatrics. 2003;112:1065-68.
Guilherme L, Kalil J. Rheumatic fever: the T-cell response leading to autoimmune aggression in the heart. Autoimmune Rev. 2002;1:261-66.
Cunningham MW. Autoimmunity and molecular mimicry in the pathogenesis of post-streptococcal heart disease. Front Biosci. 2003;8:s533-43.
Bisno AL, Brito MO, Collins CM. Molecular basis of Group A streptococcal virulence. Lancet Infect Dis. 2003;3:191-200.
Stollerman GH. Current issues in the prevention of rheumatic fever. Minerva Med. 2002;93:371-87.
Benoist C, Mathis D. Autoimmunity provoked by infection: how gfood is the case for epitope mimicry. Nat Immunol. 2001;2:797-801.
FROM THE INTERNET
Werner K. Medical Encyclopedia: Rheumatic fever. MedlinePlus. Update Date: 8/11/2003. 3pp.
Mayo Clinic Staff. Rheumatic fever. MayoClinic.com. April 16, 2003. 4pp.
American Heart Association. Rheumatic Heart Disease / Rheumatic Fever. nd. 2pp.
Rheumatic Fever. Texas Heart Institute. Updated July 2004. 3pp.
Report last updated: 2009/04/07 00:00:00 GMT+0