Rheumatic Fever

Rheumatic Fever

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Rheumatic Fever is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Acute Rheumatic Fever
  • Inflammatory Rheumatism
  • Rheumatic Arthritis

Disorder Subdivisions

  • None

General Discussion

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.

Symptoms

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.

Causes

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.

Affected Populations

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.

Standard Therapies

Diagnosis

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

Fever

Prior RF or evidence of rheumatic heart disease

Abnormal ECG

Positive blood test for inflammation

New heart murmors



Treatment

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.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

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.



REVIEW ARTICLES

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.

www.nlm.nih.gov/medlineplus/ency/article/003940.htm



Mayo Clinic Staff. Rheumatic fever. MayoClinic.com. April 16, 2003. 4pp.

www.mayoclinic.com/invoke.cfm?id=DS00250



American Heart Association. Rheumatic Heart Disease / Rheumatic Fever. nd. 2pp.

www.americanheart.com/presenter.jhtml?identifier=4709



Rheumatic Fever. Texas Heart Institute. Updated July 2004. 3pp.

www.tmc.edu/thi/rheufev.html

Resources

American Autoimmune Related Diseases Association, Inc.

22100 Gratiot Ave.

Eastpointe, MI 48021

Tel: (586)776-3900

Fax: (586)776-3903

Tel: (800)598-4668

Email: aarda@aarda.org

Internet: http://www.aarda.org/



Centers for Disease Control and Prevention

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Atlanta, GA 30333

Tel: (404)639-3534

Tel: (800)232-4636

TDD: (888)232-6348

Email: cdcinfo@cdc.gov

Internet: http://www.cdc.gov/



NIH/National Institute of Allergy and Infectious Diseases

Office of Communications and Government Relations

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Tel: (301)496-5717

Fax: (301)402-3573

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Email: ocpostoffice@niaid.nih.gov

Internet: http://www.niaid.nih.gov/



World Health Organization (WHO)

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Tel: 41227912111

Fax: 41227913111

Internet: http://www.who.int/en/



Genetic and Rare Diseases (GARD) Information Center

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Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



AutoImmunity Community

Email: moderator@autoimmunitycommunity.org

Internet: http://www.autoimmunitycommunity.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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