Rheumatic fever

Definition

Rheumatic fever (RF) is an illness that arises as a complication of untreated or inadequately treated strep throat infection. Rheumatic fever can seriously damage the valves of the heart.

Description

Throat infection with a member of the Group A streptococcus (strep) bacteria is a common problem among school-aged children. It is easily treated with a 10-day course of antibiotics by mouth. However, when such a throat infection occurs without symptoms, or when a course of medication is not taken for the full ten days, there is a 3 percent chance the person will develop rheumatic fever. Other types of strep infections (such as of the skin) do not put the patient at risk for RF.

Demographics

Children between the ages of five and 15 are most susceptible to strep throat, and therefore most susceptible to rheumatic fever. Other risk factors include poverty, overcrowding (as in military camps), and lack of access to good medical care. Just as strep throat occurs most frequently in fall, winter, and early spring, so does rheumatic fever. Rheumatic fever used to be a leading cause of death and disability in children. Since 1960, it has become much less common in the United States, partially because of increasingly accurate and swift diagnosis of strep throat. It is still a large problem in many developing countries. Moreover, children who have family members who have had rheumatic fever are more likely to get rheumatic fever themselves.

Causes and symptoms

Two different theories exist about how a bacterial throat infection can develop into rheumatic fever. One theory suggests that the bacteria produce some kind of poisonous chemical (toxin). This toxin is sent into circulation throughout the bloodstream, thus affecting other systems of the body.

Research seems to point to a different theory, however. The second theory suggests that the disease is caused by the body's immune system acting inappropriately. The body produces immune cells (called antibodies), that are specifically designed to recognize and destroy invading agent—in this case, streptococcal bacteria. The antibodies are able to recognize the bacteria because the bacteria contain special markers called antigens. Due to a resemblance between Group A streptococcus bacteria's antigens and antigens present on the body's own cells, the antibodies may mistakenly attack the body itself.

It is interesting to note that members of certain families seem to have a greater tendency to develop rheumatic fever than do others. This statistical fact could be related to the above theory, in that these families may have cell antigens that more closely resemble streptococcal antigens than do members of other families.

In addition to fever, in about 75 percent of all cases of RF one of the first symptoms is arthritis. The joints (especially those of the ankles, knees, elbows, and wrists) become red, hot, swollen, shiny, and extraordinarily painful. Unlike many other forms of arthritis, this arthritis may not occur symmetrically (affecting a particular joint on both the right and left sides, simultaneously). The arthritis of RF rarely strikes the fingers, toes, or spine. The joints become so tender that even the touch of bed sheets or clothing is terribly painful.

A particular type of involuntary movement, coupled with emotional instability, occurs in about 10 percent of all RF patients. The patient begins experiencing a change in coordination, often first noted by changes in handwriting. The arms or legs may flail or jerk uncontrollably. The patient seems to develop a low threshold for anger and sadness. This feature of RF is called Sydenham's chorea or St. Vitus' dance.

A number of skin changes are common to RF. A rash called erythema marginatum often develops (especially in those patients who will develop heart problems from their illness), composed of pink splotches that may eventually spread into each other. The rash does not itch. Bumps the size of peas may occur under the skin. These are called subcutaneous nodules. They are hard to the touch, but not painful. These nodules most commonly occur over the knee and elbow joint, as well as over the spine.

The most serious problem occurring in RF is called pancarditis ("pan" means total; "carditis" refers to inflammation of the heart). Pancarditis is an inflammation that affects all aspects of the heart, including the lining of the heart (endocardium), the sac containing the heart (pericardium), and the heart muscle itself (myocardium). About 40 to 80 percent of all RF patients develop pancarditis. This RF complication has the most serious, long-term effects. The valves within the heart (structures that allow the blood to flow only in the correct direction and only at the correct time in the heart's pumping cycle) are frequently damaged during the course of pancarditis. This effect may result in blood that either leaks back in the wrong direction or has a difficult time passing a stiff, poorly moving valve. Either way, damage to a valve can result in the heart having to work very hard in order to move the blood properly. The heart may not be able to "work around" the damaged valve, which may result in a consistently inadequate amount of blood entering the circulation.

When to call the doctor

The doctor should be contacted if the child is displaying any of the signs or symptoms of rheumatic fever. If they are not indications of rheumatic fever, they could be indicative of another disease or disorder. The doctor should also be contacted if the child has had a sore throat and fever for more than 24 hours. The doctor will do a strep test, and if the child does have strep throat the doctor can administer antibiotics that will help prevent rheumatic fever.

Diagnosis

There are no laboratory tests that can determine with complete certainty if a child has rheumatic fever. Some laboratory tests may be used in conjunction with careful examination of the patient to determine if the child has RF. A list of diagnostic criteria has been created. These "Jones Criteria" are divided into major and minor criteria. A patient can be diagnosed with RF if he or she has either two major criteria (conditions) or one major and two minor criteria. In either case, it must also be proved that the individual has had a previous infection with streptococcus.

The major criteria include:

• carditis
• arthritis
• chorea
• subcutaneous nodules
• erythema marginatum

The minor criteria include:

• fever
• joint pain (without actual arthritis)
• evidence of electrical changes in the heart (determined by measuring electrical characteristics of the heart's functioning during a test called an electrocardiogram, or EKG)
• evidence (through a blood test) of the presence in the blood of certain proteins that are produced early in an inflammatory/infectious disease

Tests are also performed to provide evidence of recent infection with group A streptococcal bacteria. A swab of the throat can be taken and smeared on a gel-like substance in a petri dish to see if bacteria will multiply and grow over 24 to 72 hours. These bacteria can then be specially processed and examined under a microscope to identify streptococcal bacteria. Other tests can be performed to see if the patient is producing specific antibodies that are only made in response to a recent strep infection.

Treatment

A 10-day course of penicillin by mouth or a single injection of penicillin G is usually the first line of treatment for RF. If the child does not tolerate or is allergic to penicillin, other antibiotics can be used effectively. These antibiotics are given to help cure a strep infection, if the child still has one. Patients will need to remain on some regular dose of antibiotic to prevent recurrence of RF. This can mean a small daily dose of antibiotic by mouth or an injection every three to four weeks. Some practitioners keep patients on this regimen for five years or until they reach 18 years of age whichever comes first. Other practitioners prefer to continue treating those patients who will be regularly exposed to streptococcal bacteria (teachers, medical workers), as well as those patients with known RF heart disease.

Arthritis quickly improves when the patient is given a preparation containing aspirin or some other anti-inflammatory agent (e.g. ibuprofen). Mild carditis also improves with such anti-inflammatory agents, although more severe cases of carditis require steroid medications. A number of medications are available to treat the involuntary movements of chorea, including diazepam for mild cases and haloperidol for more severe cases.

Prognosis

The long-term prognosis of an RF patient depends primarily on whether he or she develops carditis. This manifestation of RF is the only one that can have permanent effects. Those patients with no or mild carditis have an excellent prognosis. Those with more severe carditis have a risk of heart failure, as well as a risk of future heart problems that may lead to the need for valve replacement surgery. Patients who have had rheumatic fever are at an increased risk of getting it again.

KEY TERMS

Antibody —A special protein made by the body's immune system as a defense against foreign material (bacteria, viruses, etc.) that enters the body. It is uniquely designed to attack and neutralize the specific antigen that triggered the immune response.

Antigen —A substance (usually a protein) identified as foreign by the body's immune system, triggering the release of antibodies as part of the body's immune response.

Arthritis —A painful condition that involves inflammation of one or more joints.

Autoimmune disorder —One of a group of disorders, like rheumatoid arthritis and systemic lupus erythematosus, in which the immune system is overactive and has lost the ability to distinguish between self and non-self. The body's immune cells turn on the body, attacking various tissues and organs.

Chorea —Involuntary movements in which the arms or legs may jerk or flail uncontrollably.

Immune system —The system of specialized organs, lymph nodes, and blood cells throughout the body that work together to defend the body against foreign invaders (bacteria, viruses, fungi, etc.).

Inflammation —Pain, redness, swelling, and heat that develop in response to tissue irritation or injury. It usually is caused by the immune system's response to the body's contact with a foreign substance, such as an allergen or pathogen.

Pancarditis —Inflammation of the lining of the heart, the sac around the heart, and the muscle of the heart.

Prevention

Prevention of the development of RF involves proper diagnosis of initial strep throat infections and adequate treatment within 10 days with an appropriate antibiotic. Prevention of RF recurrence requires continued antibiotic treatment, perhaps for life. Prevention of complications of already-existing RF heart disease requires that the patient always take a special course of antibiotics when he or she undergoes any kind of procedure (even dental cleanings) that might allow bacteria to gain access to the bloodstream.

Parental concerns

Rheumatic fever can be life-threatening if not treated. It can also lead to lifelong heart problems. The best way for parents to prevent rheumatic fever is to take seriously sore throats that are accompanied with fever and to take the child to a doctor to test for strep throat. Children who have had rheumatic fever need to take extra precautions to ensure they do not have repeat attacks triggered by strep infections.

See also Strep throat.

Resources

BOOKS

Margulies, Phillip. Everything You Need to Know about Rheumatic Fever. New York: Rosen Publishing Group, 2004.

PERIODICALS

Mercadante, Marcos T., et al. "The Psychiatric Symptoms of Rheumatic Fever." American Journal of Psychiatry 157, i.12 (December 2000): 2036.

Steeg, Carl N., et al. "Rheumatic Fever: No Cause for Complacence." Patient Care 34, i.14 (July 30, 2000): 40.

Stollerman, Gene H. "Rheumatic Fever in the 21st Century." Clinical Infectious Diseases 33, no. 16 (September 15, 2001): 806.

ORGANIZATIONS

American Heart Association. 7272 Greenville Ave., Dallas, TX 75231. Web site: <www.americanheart.org>.

Tish Davidson, A.M.
Rosalyn Carson-DeWitt, MD

Rheumatic Fever

Definition

Rheumatic fever (RF) is an illness which arises as a complication of untreated or inadequately treated strep throat infection. Rheumatic fever can seriously damage the valves of the heart.

Description

Throat infection with a member of the Group A streptococcus (strep) bacteria is a common problem among school-aged children. It is easily treated with a ten-day course of antibiotics by mouth. However, when such a throat infection occurs without symptoms, or when a course of medication is not taken for the full ten days, there is a 3% chance of that person developing rheumatic fever. Other types of strep infections (such as of the skin) do not put the patient at risk for RF.

Children between the ages of five and fifteen are most susceptible to strep throat, and therefore most susceptible to rheumatic fever. Other risk factors include poverty, overcrowding (as in military camps), and lack of access to good medical care. Just as strep throat occurs most frequently in fall, winter, and early spring, so does rheumatic fever.

Causes and symptoms

Two different theories exist as to how a bacterial throat infection can develop into the disease called rheumatic fever. One theory, less supported by research evidence, suggests that the bacteria produce some kind of poisonous chemical (toxin). This toxin is sent into circulation throughout the bloodstream, thus affecting other systems of the body.

Research seems to point to a different theory, however. This theory suggests that the disease is caused by the body's immune system acting inappropriately. The body produces immune cells (called antibodies), which are specifically designed to recognize and destroy invading agents; in this case, streptococcal bacteria. The antibodies are able to recognize the bacteria because the bacteria contain special markers called antigens. Due to a resemblance between Group A streptococcus bacteria's antigens and antigens present on the body's own cells, the antibodies mistakenly attack the body itself.

It is interesting to note that members of certain families seem to have a greater tendency to develop rheumatic fever than do others. This could be related to the above theory, in that these families may have cell antigens which more closely resemble streptococcal antigens than do members of other families.

In addition to fever, in about 75% of all cases of RF one of the first symptoms is arthritis. The joints (especially those of the ankles, knees, elbows, and wrists) become red, hot, swollen, shiny, and extraordinarily painful. Unlike many other forms of arthritis, the arthritis may not occur symmetrically (affecting a particular joint on both the right and left sides, simultaneously). The arthritis of RF rarely strikes the fingers, toes, or spine. The joints become so tender that even the touch of bedsheets or clothing is terribly painful.

A peculiar type of involuntary movement, coupled with emotional instability, occurs in about 10% of all RF patients (the figure used to be about 50%). The patient begins experiencing a change in coordination, often first noted by changes in handwriting. The arms or legs may flail or jerk uncontrollably. The patient seems to develop a low threshold for anger and sadness. This feature of RF is called Sydenham's chorea or St. Vitus' Dance.

A number of skin changes are common to RF. A rash called erythema marginatum develops (especially in those patients who will develop heart problems from their illness), composed of pink splotches, which may eventually spread into each other. It does not itch. Bumps the size of peas may occur under the skin. These are called subcutaneous nodules; they are hard to the touch, but not painful. These nodules most commonly occur over the knee and elbow joint, as well as over the spine.

The most serious problem occurring in RF is called pancarditis ("pan" means total; "carditis" refers to inflammation of the heart). Pancarditis is an inflammation that affects all aspects of the heart, including the lining of the heart (endocardium), the sac containing the heart (pericardium), and the heart muscle itself (myocardium). About 40-80% of all RF patients develop pancarditis. This RF complication has the most serious, long-term effects. The valves within the heart (structures which allow the blood to flow only in the correct direction, and only at the correct time in the heart's pumping cycle) are frequently damaged during the course of pancarditis. This may result in blood which either leaks back in the wrong direction, or has a difficult time passing a stiff, poorly moving valve. Either way, damage to a valve can result in the heart having to work very hard in order to move the blood properly. The heart may not be able to "work around" the damaged valve, which may result in a consistently inadequate amount of blood entering the circulation.

Diagnosis

Diagnosis of RF is done by carefully examining the patient. A list of diagnostic criteria has been created. These "Jones Criteria" are divided into major and minor criteria. A patient can be diagnosed with RF if he or she has either two major criteria (conditions), or one major and two minor criteria. In either case, it must also be proved that the individual has had a previous infection with streptococcus.

The major criteria include:

• carditis
• arthritis
• chorea
• subcutaneous nodules
• erythema marginatum

The minor criteria include:

• fever
• joint pain (without actual arthritis)
• evidence of electrical changes in the heart (determined by measuring electrical characteristics of the heart's functioning during a test called an electrocardiogram, or EKG)
• evidence (through a blood test) of the presence in the blood of certain proteins, which are produced early in an inflammatory/infectious disease.

Tests are also performed to provide evidence of recent infection with group A streptococcal bacteria. A swab of the throat can be taken, and smeared on a substance in a petri dish, to see if bacteria will multiply and grow over 24-72 hours. These bacteria can then be specially processed, and examined under a microscope, to identify streptococcal bacteria. Other tests can be performed to see if the patient is producing specific antibodies; that are only made in response to a recent strep infection.

Treatment

A 10-day course of penicillin by mouth, or a single injection of penicillin G-is the first line of treatment for RF. Patients will need to remain on some regular dose of penicillin to prevent recurrence of RF. This can mean a small daily dose of penicillin by mouth, or an injection every three weeks. Some practitioners keep patients on this regimen for five years, or until they reach 18 years of age (whichever comes first). Other practitioners prefer to continue treating those patients who will be regularly exposed to streptococcal bacteria (teachers, medical workers), as well as those patients with known RF heart disease.

Arthritis quickly improves when the patient is given a preparation containing aspirin, or some other anti-inflammatory agent (ibuprofen). Mild carditis will also improve with such anti-inflammatory agents, although more severe cases of carditis will require steroid medications. A number of medications are available to treat the involuntary movements of chorea, including diazepam for mild cases, and haloperidol for more severe cases.

Prognosis

The long-term prognosis of an RF patient depends primarily on whether he or she develops carditis. This is the only manifestation of RF which can have permanent effects. Those patients with no or mild carditis have an excellent prognosis. Those with more severe carditis have a risk of heart failure, as well as a risk of future heart problems, which may lead to the need for valve replacement surgery.

Prevention

Prevention of the development of RF involves proper diagnosis of initial strep throat infections, and adequate treatment within 10 days with an appropriate antibiotic. Prevention of RF recurrence requires continued antibiotic treatment, perhaps for life. Prevention of complications of already-existing RF heart disease requires that the patient always take a special course of antibiotics when he or she undergoes any kind of procedure (even dental cleanings) that might allow bacteria to gain access to the bloodstream.

Resources

ORGANIZATIONS

Centers for Disease Control and Prevention. 1600 Clifton Rd., NE, Atlanta, GA 30333. (800) 311-3435, (404) 639-3311. 〈http://www.cdc.gov〉.

KEY TERMS

Antibodies— Specialized cells of the immune system which can recognize organisms that invade the body (such as bacteria, viruses, and fungi). The antibodies are then able to set off a complex chain of events designed to kill these foreign invaders.

Antigen— A special, identifying marker on the outside of cells.

Arthritis— Inflammation of the joints.

Autoimmune disorder— A disorder in which the body's antibodies mistake the body's own tissues for foreign invaders. The immune system therefore attacks and causes damage to these tissues.

Chorea— Involuntary movements in which the arms or legs may jerk or flail uncontrollably.

Immune system— The system of specialized organs, lymph nodes, and blood cells throughout the body, which work together to prevent foreign invaders (bacteria, viruses, fungi, etc.) from taking hold and growing.

Inflammation— The body's response to tissue damage. Includes hotness, swelling, redness, and pain in the affected part.

Pancarditis— Inflammation of the lining of the heart, the sac around the heart, and the muscle of the heart.

rheumatic fever , systemic inflammatory disease, extremely variable in its manifestation, severity, duration, and aftereffects. It is frequently followed by serious heart disease, especially when there are repeated attacks. Rheumatic fever usually affects children. It is closely related to a preceding streptococcal infection (e.g., streptococcal tonsillitis or pharyngitis). Some of its symptoms are tenderness and inflammation about the joints, fever, jerky movements, nodules under the skin, and skin rash. If inflammation of the heart, or myocarditis, is mild, there is no permanent heart damage, but if the valves of the heart become inflamed, they may become scarred and deformed, permanently impairing their function. Such heart damage can sometimes be corrected by surgery.

Treatment of rheumatic fever is with penicillin, salicylates, and steroids; extended rest is usually necessary. Rheumatic fever may be prevented by prompt treatment of all streptococcal infections. Cardiac damage may possibly be avoided if prophylactic measures are taken after a first attack of rheumatic fever, i.e., long-term maintenance doses of antibiotics, to discourage streptococcal infections and recurrences of rheumatic fever. Rheumatic fever has declined in incidence in the industrialized countries, but has increased in prevalence in the Third World. See also streptococcus .

RHEUMATIC FEVER

DEFINITION

Rheumatic fever (RF; pronounced roo-MAT-ic FEE-ver) is a disease caused by a group of bacteria known as Group A streptococcus (pronounced STREP-tuh-cac-us). The exact conditions that lead to rheumatic fever are not well understood. However, the condition often follows a case of strep throat that was not treated or not treated correctly. Rheumatic fever can seriously damage the valves of the heart.

DESCRIPTION

Streptococcal infections of the throat and ear are common in young children. These infections can be treated efficiently with antibiotics. When so treated, the infections clear up in about a week. Patients seldom experience complications.

Some streptococcal infections are very mild, however. Children may experience no symptoms, so their parents do not seek medical advice. Also, patients sometimes do not take their full course of medication. They stop taking pills when their symptoms are gone. But streptococcal bacteria may still be present. In either of these cases, complications may develop. One of the most serious complications is rheumatic fever.

Children between the ages of five and fifteen are at greatest risk for RF. The rate of RF in the United States has dropped dramatically. Today, it is rarely seen in this country. It continues to be widespread in developing nations, however. Poverty, overcrowding, and lack of access to proper medical care contribute to the high rate of RF in these areas.

CAUSES

There is still some debate as to how rheumatic fever develops. The most popular theory is that the condition is caused by the body's own immune system. The immune system consists of organs, tissues, cells, and chemicals designed to attack foreign invaders, such as bacteria or viruses. One way the immune system protects the body is with antibodies. Antibodies are chemicals produced to fight off specific foreign agents. There is a specific type of antibody, for example, for every bacterium and virus that can invade the human body.

But the immune system sometimes makes mistakes and produces antibodies that attack the body's own cells. The antibodies kill cells and cause a type of disease known as an autoimmune ("against itself") disorder. Rheumatic fever is thought to be an autoimmune disorder (see autoimmune disorder entry).

SYMPTOMS

In addition to fever, there are five main symptoms of RF. One symptom is arthritis (see arthritis entry). Arthritis is an inflammation of the joints that causes severe pain and swelling. In the case of RF, arthritis produces red, hot, swollen, shiny, and very painful joints of the ankles, knees, elbows, and wrists. The joints may become so sore that even the touch of bed sheets or clothing is too painful to endure.

A second symptom of RF is chorea (pronounced co-REE-uh). Chorea is a disorder of the nervous system. It causes a patient to lose coordination and develop jerky movements of the arms and legs. The patient may also develop emotional problems. He or she may become angry or sad for no reason at all. The chorea that occurs as a result of RF is called Syndenham's chorea. At one time, the condition was known as St. Vitus's Dance.

A third symptom of RF is a rash. The rash consists of pink splotches on the body that are not itchy. The medical term for this rash is erythema marginatum (pronounced air-uh-THEE-muh MAR-gin-ay-tum). Along with the rash there may be small bumps just under the skin. The bumps are hard to the touch but not painful. They occur most commonly over the knee and elbow joints. The bumps constitute the fourth symptom of RF. They are also known as subcutaneous nodules (pronounced sub-CYOO-taynee-us NOD-yools).

Rheumatic Fever: Words to Know

Antibodies:

Chemicals produced by the body's immune system to fight off very specific foreign invaders, such as bacteria and viruses.

Arthritis:

Inflammation of the joints.

Autoimmune disorder:

A medical condition in which the body's immune system mistakes the body's own tissues for foreign invaders and attempts to destroy those tissues.

Carditis:

Inflammation of the heart.

Chorea:

Involuntary movements that may cause the arms or legs to jerk about uncontrollably.

Immune system:

A system of organs, tissues, cells, and chemicals that work together to fight off foreign invaders, such as bacteria and viruses.

Inflammation:

The body's response to tissue damage that includes heat, swelling, redness, and pain.

The fifth and most serious symptom of rheumatic fever is called carditis (pronounced car-DIE-tis). The term "carditis" means an inflammation of the heart. About 40 percent to 80 percent of all patients with rheumatic fever develop carditis. It is the most serious complication associated with RF.

One common effect of carditis is damage to the heart valves. The heart valves are flaps of tissue that control the flow of blood in the heart. They ensure that blood always flows in the right direction as it passes through the heart.

If heart valves are damaged, one of two things can happen. First, the flow of blood reverses direction. Second, the valves become so stiff that it is difficult for blood to get through them. Either way, the heart has to work unusually hard to keep blood moving properly. In some cases, the heart has to work so hard that it becomes weakened or fails. The patient develops chronic (long-term) heart disease or dies of a heart attack.

DIAGNOSIS

Diagnosis of rheumatic fever can be difficult because patients may have one, two, or more of the five symptoms listed above. The five symptoms for which a doctor looks include arthritis, chorea, carditis, erythema marginatum, and subcutaneous nodules. Fever is also present. However, fever by itself is not of much use in making a diagnosis. Many kinds of medical disorders have fever as a symptom.

GERHARD DOMAGK

Gerhard Domagk was a German biochemist who was born on October 30, 1895. He earned his medical degree in 1921 and then took a job with a large company, Farbenindustrie. Farbenindustrie manufactured industrial dyes.

Domagk was interested in finding out whether any of the dyes produced by Farbenindustrie had biological effects on animals. In one study, he injected a dye called Prontosil Red into a group of experimental mice. The mice had been given a streptococcus infection. Domagk was amazed to discover that the mice were cured of their disease by the dye.

That discovery might have lead nowhere except for a terrible event in Domagk's life. His daughter Hildegarde pricked herself with a knitting needle and developed a streptococcal infection herself. In desperation, Domagk injected Prontosil Red into his daughter. Again, the dye seemed to work miracles. Hildegarde quickly recovered from the disease.

Shortly after these events, the French chemist Daniele Bovet explained how Prontosil Red works. He showed that only one part of the compound was involved in killing bacteria. That part was a molecule called sulfanilamide. Sulfanilamide was the first of a large group of related compounds, called sulfanamides (or "sulfa drugs") to be used against bacterial infections.

A number of laboratory tests are also used to diagnose RF. For example, a throat smear can be taken to look for the presence of streptococcal bacteria. In a throat smear, a cotton swab is rubbed across the back of the patient's

throat. The material collected on the swab is then placed in a warm, moist environment for twenty-four to seventy-two hours. Any bacteria present in the material begin to grow during that period. If streptococcal bacteria are present, they can be seen with a microscope.

Laboratory tests can also be used to look for antibodies to the streptococcal bacteria. Blood taken from the patient's arm can be treated with chemicals that make these antibodies show up. The presence of the antibodies means that the patient has been infected with the streptococcal bacteria.

An electrocardiogram (ECG; pronounced ih-LEK-tro-KAR-dee-o-gram) can also be used to diagnose RF. An electrocardiogram is a test that measures the electrical activity of the heart. If the heart has been damaged by carditis, its electrical rhythms will be different from those of a healthy heart.

All of these symptoms and tests taken together can provide a diagnosis of rheumatic fever.

TREATMENT

Rheumatic fever is usually treated with penicillin. The patient usually receives a single injection or a ten-day course of pills. Doctors often prescribe a regular dose of penicillin after the symptoms of RF disappear. This program helps prevent the return of RF. Other patients who continue to receive regular doses of penicillin are those individuals who may come into contact with the streptococcal bacterium. This group includes teachers and medical workers, as well as patients with known RF heart disease.

The symptoms of arthritis can be treated with aspirin, acetaminophen (Tylenol), or ibuprofen (Motrin, Advil). These same drugs can be used to treat mild cases of carditis. Aspirin should not be given to children with a fever because it may cause the serious illness Reye's syndrome (see Reye's syndrome entry). More severe cases of carditis are treated with steroid medications. Chorea is treated with a variety of sedatives and tranquilizers that help relax the patient.

PROGNOSIS

RF patients who do not develop carditis have an excellent prognosis. Other symptoms of the disease eventually disappear without complications. Even patients who have mild cases of carditis are likely to experience a full recovery. Severe cases of carditis are another matter. In this case, patients are likely to develop a variety of heart problems that may lead to heart failure. Serious heart problems can often be treated by surgery.

PREVENTION

Rheumatic fever can be prevented with early and proper diagnosis of streptococcal infections of the throat and ears. If antibiotics are given within ten days, the risk of the infection's developing into rheumatic fever is very low.

People who have had rheumatic fever are at risk for contracting the disease again. To prevent the condition from returning, a patient may have to stay on antibiotics for a long time—perhaps for life. People who already have heart damage resulting from RF can prevent further complications. The usual procedure is for the patient to take antibiotics any time he or she might be exposed to the streptococcal bacterium.

FOR MORE INFORMATION

Books

Stoffman, Phyllis. The Family Guide to Preventing and Treating 100 Infectious Diseases. New York: John Wiley and Sons, Inc. 1995.

Web sites

"Rheumatic Fever." Yahoo! Health. [Online] http://dir.yahoo.com/Health/Diseases_and_Conditions/Rheumatic_Fever (accessed on November 5, 1999).

"Rheumatic fever." HealthAnswers Medical Reference Library. [Online] http://www.healthanswers.com/adam/top/view.asp?filename=003940.htm&rdir (accessed on November 5, 1999).

rheumatic fever (RF, acute rheumatism) (roo-mat-ik) n. a disease affecting mainly children and young adults that arises as a delayed complication of infection of the upper respiratory tract with haemolytic streptococci. The main features are fever, arthritis, Sydenham's chorea, and inflammation of the heart muscle, its valves, and the membrane surrounding the heart. The infection is treated with antibiotics (e.g. penicillin) and bed rest. chronic rheumatic heart disease a complication of rheumatic fever, in which there is scarring and chronic inflammation of the heart and its valves leading to heart failure, murmurs, and damage to the valves.

Rheumatic Fever

From a Sore Throat to a Damaged Heart

A Turn for the Worse

The Importance of Antibiotics

Rheumatic (roo-MA-tik) fever is a complication of a strep throat infection that can lead to permanent heart damage and death. It is most common in children.

KEYWORDS

for searching the Internet and other reference sources

Heart disease

Streptococcal infection

Sydenham’s chorea

Until recently, doctors thought rheumatic fever had almost disappeared from the United States. In 1950, before the widespread use of antibiotics to fight strep* infections, more than 22,000 people died of rheumatic fever and the heart disease it caused. During the 1950s, almost 25 of every 100,000

* strep throat

is a contagious sore throat caused by a strain of bacteria known as Streptococcus.

The Discovery Of Aspirin

In the mid-nineteenth century, the Reverend Edmund Stone unwittingly discovered the earliest known effective treatment for rheumatic fever and other conditions characterized by rheumatism. Stone, like many physicians of his time, believed that God grew healing herbs for specific diseases in the localities where they naturally ocurred. Willing to put his idea to the test, he administered willow bark, which he himself sampled, to some 50 persons suffering with rheumatic fever. He reported effective results in each case. The bark was later found to contain an active ingredient of salicin (SAL-i-sin), first extracted and analyzed by Dr. Thomas MacLagan in 1839. Other chemists later produced the salicylate (sal-i-SY-late) group of drugs, which yielded sodium salicylate in 1899. This drug came to be known as aspirin and became useful for remedying symptoms associated with rheumatic fever as well as a general pain reliever.

Americans got rheumatic fever each year. But as use of antibiotics such as penicillin became more common in the 1960s, and as more poor children had access to better medical care, rheumatic fever became rare.

By the early 1980s, only about 1 in every 100,000 Americans developed it. But by 1985, the disorder had re-emerged as a significant problem in some communities. There were outbreaks in Salt Lake City, New York, Dallas, San Diego, Akron, and Columbus.

Doctors were puzzled and have renewed their interest in fighting rheumatic fever. The number of cases remains small in the United States, although in poor, less developed countries, rheumatic fever is a significant problem. Doctors are not sure if the fever’s comeback in the United States is temporary, but it has shown that everyone needs to be watchful for the effects of strep infections.

From a Sore Throat to a Damaged Heart

Rheumatic fever sometimes results when the body’s immune system reacts to infection by a bacterium known as Group A Streptococcus, commonly called strep. The same bacteria that cause strep throat can lead to other disorders, such as scarlet fever.

When the body becomes infected with the strep bacteria, the immune system produces antibodies to fight the infection. Rheumatic fever results when these antibodies begin to affect other parts of the body instead of just fighting the infection. The antibodies react to organs such as the heart as if they were the strep bacteria, perhaps because parts of these organs are chemically similar to strep.

Doctors are not sure exactly why some strep infections develop into rheumatic fever and others do not. The disorder occurs most often in children between ages 5 and 15, although it can strike younger children and adults, too.

A Turn for the Worse

The first signs of rheumatic fever usually occur within several weeks after a strep throat infection. Sometimes, people appear to have recovered from the sore throat but suddenly begin to show other symptoms:

• Muscle aches and joint pain and swelling resembling arthritis. The pain usually moves from one joint to another.
• Fever, vomiting, and sometimes nosebleeds.
• A red rash, especially on the chest, arms, and legs, which might disappear in a few hours. Lumps below the skin also may occur.
• Fatigue and problems breathing, because the heart is affected. The heartbeat also may be abnormal.
• Sydenham’s chorea (see sidebar), which is uncontrollable twitching and body movements.

The most dangerous consequence of rheumatic fever is inflammation and weakening of the heart muscle. The valves that control passage of blood in and out of the heart can be damaged so that they fail to open and close properly. This condition is called rheumatic heart disease.

Sydenham’s Chorea

Sydenham’s chorea is the name for the involuntary movements and twitching that some rheumatic fever patients display.

”Chorea” (pronounced like the country Korea) comes from the Greek word for “dance.” During the Middle Ages, chorea was the term used to describe people who traveled to the shrine of St. Vitus in what is now Germany. Some of the people apparently suffered from conditions involving abnormal body movements, such as epilepsy, and hoped to be healed at the shrine. (Catholics consider St. Vitus the patron saint of those with epilepsy, as well as of dancers and actors.)

Dr. Thomas Sydenham, a prominent physician in England during the 1600s, used the term “chorea” in connection with infectious diseases such as scarlet fever. Later, when rheumatic fever also was connected with strep infection, Sydenham’s chorea was the term used to describe the shaking of the upper limbs and face caused by swelling of the brain.

Sydenham’s chorea is sometimes called St. Vitus’ dance.

The Importance of Antibiotics

A doctor may suspect a strep infection if a patient with a sore throat also has a fever and severe headache. However, the symptoms and physical exam findings in people with strep throat are very similar to those in people with sore throat due to a virus infection or other cause. Therefore, strep infections must be confirmed with laboratory tests. Doctors use a cotton swab to wipe the throat to test for the strep bacteria.

If the infection is caused by strep, the doctor will usually prescribe an antibiotic such as penicillin for 10 days. Doctors say that it is important to take all the antibiotic prescribed, even if the symptoms of the strep infection disappear.

Not all untreated strep infections lead to complications like rheumatic fever. For people who get rheumatic fever, doctors use antibiotics as well as other drugs that reduce swelling and relieve pain. They also closely watch the heart, to ensure that there are no problems with blood flowing through it. If the heart valves are damaged, surgery might be necessary to fix one or more valves.

The best way to avoid rheumatic fever is to treat strep infection promptly with antibiotics. Doctors, however, are worried that some bacteria are becoming resistant to traditional antibiotics. Research is ongoing into the best ways to use antibiotics and to develop new drugs to fight infections also.

See also

Arthritis

Fever

Heart Disease

Strep Throat

rheumatic fever Inflammatory disorder characterized by fever and painful swelling of the joints. Rare in the modern developed world, it mostly affects children and young adults. An important complication is possible damage to the heart valves, leading to rheumatic heart disease in later life.

rheu·mat·ic fe·ver • n. a noncontagious acute fever marked by inflammation and pain in the joints. It chiefly affects young people and is caused by a streptococcal infection.