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$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.