$Unique_ID{BRK04188} $Pretitle{} $Title{Rheumatic Fever} $Subject{Rheumatic Fever Acute Rheumatic Fever Rheumatic Arthritis Inflammatory Rheumatism Juvenile Rheumatoid Arthritis } $Volume{} $Log{} Copyright (C) 1987, 1988, 1989, 1991 National Organization for Rare Disorders, Inc. 469: Rheumatic Fever ** IMPORTANT ** It is possible the main title of the article (Rheumatic Fever) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Acute Rheumatic Fever Rheumatic Arthritis Inflammatory Rheumatism Information on the following disease can be found in the Related Disorders section of this report: Juvenile Rheumatoid Arthritis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Rheumatic Fever is an inflammatory syndrome that can occur following streptococcal infections of the throat (strep throat). Patients initially experience moderate fever, a general feeling of ill health (malaise), a sore throat, and fatigue. Major complications can include heart disease, joint pain and arthritis, involuntary abrupt limb movements with characteristic grimaces (chorea), and possible skin symptoms. Treatment should begin as soon as possible, and be maintained for months or even years to help control serious complications. Rheumatic fever can be avoided if strep throat is vigorously treated and cured with antibiotics. Symptoms Rheumatic Fever is preceded by a streptococcal throat infection, which may or may not be noticeable as a sore throat. After a latent period of two or three weeks, the patient may develop the initial symptoms of Rheumatic Fever. The most common symptom is arthritis, which may be acutely painful. The knees are most often affected, although several joints may be involved; sometimes the inflammation may shift from joint to joint. The arthritis may completely disappear, even without treatment, usually within six weeks. The most serious problem that can be caused by Rheumatic Fever is rheumatic heart disease. Physicians must carefully monitor the heart throughout the course of the disorder. 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. (For more information on this disorder, see "Rheumatic Fever: Down But Not Out" in the Prevalent Health Conditions/Concerns area of NORD Services.) Chorea is a rare complication of Rheumatic Fever consisting of involuntary, abrupt, nonrepetitive limb movements and characteristic grimaces. It typically occurs months after the initial streptococcal infection. Inappropriate crying or laughing, and extreme weakness may also occur. Speech is often halting, jerky, or slurred. These symptoms may disappear within a few weeks or months. 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. A skin rash (erythema marginatum) may develop, described as painless, short-term, non-itching, spotted, pink, circular in shape, resembling smoke rings that expand while clearing at the center. This rash is limited to the skin of the trunk and nearby parts of the limbs. It can last for hours or days, and may recur. Arthritis, Carditis, and Chorea are complications of Rheumatic Fever that can occur singly or in combination. Subcutaneous nodules and erythema marginatum are rarely seen without Carditis. Moderate fever, a general feeling of discomfort (malaise), and fatigue usually occur, especially when Carditis is present. Patients may also experience nosebleeds. 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. In the absence of proper treatment, severe complications may occur and progressively severe recurrences of Rheumatic Fever may develop. Affected Population Rheumatic Fever usually affects children between five and fifteen years of age, but may occur among young adults as well. Although outbreaks have steadily declined since the end of World War II in the United States, 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. Recently a reoccurrence of Rheumatic Fever developed in a number of US states. This time the disease is occurring in suburban and rural areas instead of inner city areas, and, instead of affecting poor Whites and Blacks, the disorder is affecting a white middle-class population. Related Disorders 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). Therapies: Standard Timely treatment of the group A streptococcal throat infection (strep throat) which precedes Rheumatic Fever is vitally important to prevent this disorder. Aggressive treatment of strep throat with antibiotics usually guarantees that Rheumatic Fever will not develop. Rarely, there may be no apparent symptoms of strep throat (such as soreness) to warn a parent that a child needs medical care. The subsequent lack of treatment may lead to the development of Rheumatic Fever. When Rheumatic Fever develops, it is treated with anti-inflammatory drugs to help ease the arthritic symptoms. In cases involving the heart, steroid drugs such as prednisone may be helpful. If the heart becomes enlarged (cardiomegaly), corticosteroid drugs may also prove effective to control fever, discomfort, and irregular heartbeat. In extremely severe cases, intravenous methylprednisone followed by oral prednisone may control heart inflammation. Aspirin may be prescribed to control recurrence of inflammation after steroid (prednisone) therapy is discontinued. Continued administration of antibiotics for months or years can also help avoid recurrent acute attacks. Recurrences are most likely during the first three to five years after the initial symptoms of Rheumatic Fever appear. In patients who develop rheumatic heart disease, medication may be continued well into adult life, past the age where a patient is exposed to school age children. Therapies: Investigational Researchers at Rockefeller University, New York City, have identified two monoclonal antibodies that can possibly be used to detect potential Rheumatic Fever patients. However, more research is needed before this screening method can be used more generally as a preliminary step leading go immunization of susceptible persons against strep throat and/or Rheumatic Fever. This disease entry is based upon medical information available through October 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Rheumatic Fever, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Allergy and Infections Diseases (NIAID) 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 639-3534 References RESURGENCE OF ACUTE RHEUMATIC FEVER IN THE INTERMOUNTAIN AREA OF THE UNITED STATES: L.G. Veasy, et al.; N Eng J Med (February 19, 1987, issue 316 (8)). Pp. 421-427. RHEUMATIC FEVER IN THE EIGHTIES: M. Markowitz; Pediatr Clin North Am (October 1986, issue 33(5)). Pp. 1141-1150. RHEUMATIC FEVER: DOWN BUT NOT OUT: Evelyn Zamula; FDA Consumer (July- August 1987). Pp. 26-28.