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.

— Rosalyn Carson-DeWitt, MD

An illness that follows an upper respiratory infection with the group A streptococcus (Streptococcus pyogenes) and is characterized by inflammation of the joints (arthritis) and the heart (carditis). Arthritis typically involves multiple joints and may migrate from one joint to another. The carditis may involve the outer lining of the heart, the heart muscle itself, or the inner lining of the heart. A minority of affected individuals also develop a rash (erythema marginatum), nodules under the skin, or Sydenham's chorea (a neurologic disorder characterized by involuntary, uncoordinated movements of the legs, arms, and face). Damage to heart valves may be permanent and progressive, leading to severe disability or death from rheumatic heart disease years after the initial attack. The disease occurs an average of 19 days after the infection and is thought to be the result of an abnormal immunologic reaction to the group A streptococcus. Initial attacks of rheumatic fever generally occur among individuals aged 5 to 15. Those who have had one attack are highly susceptible to recurrences after future streptococcal infections.

Initial attacks of rheumatic fever can be prevented by treatment of strep throat with penicillin for at least 10 days. Patients who have had an episode of rheumatic fever should continue taking antibiotics for many years to prevent group A streptococcal infections that may trigger a recurrence of rheumatic fever. See also Heart disorders; Streptococcus.

A severe, apparently infectious disease produced by hemolytic streptococci organisms or associated with their presence in the body; characterized by upper respiratory tract inflammation, cervical lymphadenopathy and lymphadenitis, polyarthritis, cardiac involvement, and subcutaneous nodules. The disease may be produced by an autoantibody reaction.

Definition

Rheumatic fever (RF) is an illness that occurs as a complication of untreated or inadequately treated strep throat infection. Rheumatic fever causes inflammation of tissues and organs and can result in serious damage to the heart valves, joints, central nervous system and skin.

Rheumatic fever is rare in the United States, though there were outbreaks in both New York City and in Utah in the 1990s. The disease is more prevalent in the developing world, where rheumatic fever is the leading cause of heart disease. In some countries, as many as one to two percent of children are afflicted with the disease.

Description

Though the exact cause of rheumatic fever is unknown, the disease usually follows the contraction of a throat infection caused by a member of the Group A streptococcus (strep) bacteria (called strep throat). The streptococcus A bacteria has also been linked to many serious diseases, including "flesh-eating" disease and toxic shock syndrome. About 9,700 cases of invasive diseases linked to strep A were reported in the United States in 1997. Rheumatic fever may occur in people of any age, but is most common in children between the ages of five and 15. Poverty, overcrowded living conditions, and inadequate access to medical care increase the likelihood of contracting the disease.

The initial strep throat is easily treated with a 10-day course of antibiotics taken orally. However, when a throat infection occurs without symptoms, or when a patient neglects to take the prescribed medication for the full 10-day course of treatment, there is up to an estimated 3% chance that he or she will develop rheumatic fever. Other types of strep infections (such as of the skin) do not put the patient at risk for RF.

Causes & Symptoms

Two different theories exist as to how a bacterial throat infection can result in 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 more strongly supports the theory that the disease is caused by an interaction between antibodies produced to fight the group A streptococcus bacteria and the heart tissue. The body produces immune cells (antibodies), that are specifically designed to recognize and destroy invading agents. The antibodies are able to recognize the bacteria because the bacteria contain special markers called antigens on their surface. 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, specifically heart muscle.

In 2002, a report announced that scientists had mapped the genome (genetic material) of an A streptococcus bacterium responsible for acute rheumatic fever. The discovery will help researchers map the factors in the strain of bacterium that help it overcome the body's defenses.

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 that more closely resemble streptococcal antigens than do members of other families.

Symptoms of rheumatic fever usually begin one to six weeks after a sore throat. Symptoms may include fatigue and fever, stomach pain, and vomiting. 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 extremely painful. Unlike many other forms of arthritis, symptoms may not occur symmetrically (affecting a particular joint on both the right and left sides, simultaneously). Rather, pain may move from joint to joint. 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 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 in rheumatic fever patients. A rash called erythema marginatum develops (especially in those patients who will develop heart problems from their illness), which takes the form of pink splotches that may eventually spread into each other. The rash does not itch. Bumps the size of peas or larger 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 result of 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). Heart damage caused by RF 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, which may result in blood leaking back in the wrong direction, or being unable to pass a stiff, poorly moving valve. Damage to a valve can result in the heart having to work very hard in order to circulate the blood. 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. About 40-80% of all RF patients develop a form of carditis. Heart damage, however, may not be apparent until months or years after a bout with rheumatic fever. The effect of the disease on the heart also depends on the avoidance of recurrences. The severity of heart damage is often related to the number of attacks of RF a patient experiences.

Diagnosis

The initial description of diagnostic criteria for RF were created by William Cheadle in 1889, during a virulent outbreak of the disease in London. In the 1950s, T. Duckett Jones created a list of both major and minor diagnostics for RF. According to the "Jones Criteria," a patient can be diagnosed with RF if he or she exhibits either two major criteria (conditions), or one major and two minor criteria. In either case, it must also be proven 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. The doctor may swab the throat and grow a culture to see if the bacteria will grow and multiply. The culture will be processed and examined to identify streptococcal bacteria. Blood tests can be performed to see if the patient is producing antibodies only made in response to a recent strep infection. A doctor may also do an electrocardiogram in order to check for abnormalities in the heartbeat. An echocardiogram, or ultrasound test, may be ordered to check the heart vales, cardiac function and the heart's structure.

Treatment

Though there are no proven effective alternative remedies for rheumatic fever itself, alternative methods may help patients with the results and symptoms of the disease, such as pain relief and improved cardiac function. Rheumatoid arthritis can be treated with a number of alternative therapies:

• Massage: A massage therapist uses gentle strokes to stimulate circulation in and around the joints.
• Aromatherapy: Often combined with massage, the essential oils of rosemary, benzoin, German chamomile, camphor, juniper, or lavender are used to help relieve pain. Oils of cypress, fennel, lemon, and wintergreen may be used to detoxify or reduce inflammation.
• Acupuncture: Uses small needles to stimulate appropriate acupoints for pain relief.
• Osteopathy: Recommends stretching and trigger point therapy to improve mobility, as well as craniofacial massage.

Allopathic Treatment

Penicillin is still the most effective treatment for rheumatic fever. 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.

Of major concern to medical professionals is compliance in taking oral penicillin. A full course of penicillin must be taken to prevent rheumatic fever. However, it is not always easy for patients to follow such a strict regimen. Researchers have found that the time-honored practice of thrice-daily dosing may be unnecessary. Research has shown that twice-daily dosing is just as effective as more frequent doses, and compliance may be improved, since both doses can be administered at home.

Arthritis typically improves quickly 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.

Expected Results

The long-term prognosis of an RF patient depends primarily on whether he or she develops carditis. This is the only manifestation of RF that can have permanent effects. Those patients with mild or no 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

Initial prevention of rheumatic fever depends upon prompt medical attention. Patients should see a physician if they have sore throat that lasts for more than 24 hours and is accompanied by fever. Treatment of a streptococcal throat infection with an appropriate antibiotic will usually prevent the development of rheumatic fever. Prevention of RF recurrence requires continued antibiotic treatment, perhaps for life. Prevention of complications of already-existing RF heart disease require 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.

Because of the prevalence of the Strep A bacteria, it is difficult to completely eradicate rheumatic fever. However, progress in identifying a genetic marker for predisposition to the disease and in mapping the virulence (ability to overcome the body's defenses) or the bacteria that lead to rheumatic fever may help lead to a vaccine. Researchers are also seeking to develop a rapid test for strep which would mean earlier detection and more prompt treatment of strep. In addition, in 1999, testing began on a vaccine against group A streptococcus. The development of such a vaccine was halted in the 1970s after children who received the experimental vaccine developed rheumatic fever. In 1979, the Food and Drug Administration (FDA) prohibited group A strep vaccines from ever being licensed for use, the only vaccine to carry such a prohibition. Clinical trials have been approved by the FDA, however, it will be several years before a vaccine is approved. Doctors at the National Institute of Allergy and Infectious Diseases remain hopeful that a vaccine will someday be available.

Resources

Books

Kaplan, Edward L. "Rheumatic Fever." In Harrison's Principles of Internal Medicine. edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.

Ryan, Kenneth. "Streptococci." In Sherris Medical Microbiology: An Introduction to Infectious Diseases. Norwalk, CT: Appleton and Lange, 1994.

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

Todd, James. "Rheumatic Fever." In Nelson Textbook of Pediatrics. edited by Richard Behrman. Philadelphia: W.B. Saunders Co., 1996.

Woodham, Anne and Dr. David Peters. Dorling Kindersley Encyclopedia of Healing Therapies. New York: Dorling Kindersley, 1997.

Periodicals

Albert, Daniel A., et al. "The Treatment of Rheumatic Carditis: A Review and Meta-Analysis." Medicine 74, no. 1 (January 1995): 1+.

Bass, James W., Donald A. Person, and Debora S. Chan. "Twice Daily Oral Penicillin for Treatment of Streptococcal Pharyngitis: Less is Best." Pediatrics 2000 105 (February, 2000): 423-424.

Capizzi, Stephen A., et al. "Rheumatic Fever Revisited: Keep This Diagnosis on Your List of Suspects." Postgraduate Medicine 102, no. 6 (December 1997): 65+.

Eichbaum, Q.G., et al. "Rheumatic Fever: Autoantibodies Against a Variety of Cardiac, Nuclear, and Streptococcal Antigens." Annals of the Rheumatic Diseases 54, no. 9 (September 1995): 740+.

Harder, B. "Deciphering Virulence: Heart-Harming Bacteria Flaunt Unique Viral Genes." Science News 161, no. 13 (March 30, 2002): 197.

Markowitz, Milton. "Rheumatic Fever: A Half-Century Perspective." Pediatrics (July, 1998): 272-275.

Pollack, Andrew. "In the Works: Tests on Strep Vaccine Restart, Gingerly." The New York Times (July 20, 1999): Section F, Page 7, Column 1.

"Rheumatic Fever." In Clinical Reference Systems (July 1, 1999): 1264.

Stollerman, Gene H. "Rheumatic Carditis." Lancet 346, no. 8972 (August 12, 1995): 390+.

Stollerman, Gene H. "Rheumatic Fever." Lancet (March 29, 1997) : 935 - 943.

Organizations

Centers for Disease Control and Prevention. (404) 332-4559. http://www.cdc.gov.

[Article by: Amy Cooper; Teresa G. Odle]

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.

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.

[Article by: Tish Davidson, A.M.
Rosalyn Carson-DeWitt, MD]

For more information on rheumatic fever, visit Britannica.com.

An infectious disease occurring most often in children who have had a previous infection with a strain of streptococcus. Rheumatic fever, which is characterized by fever and joint pain, can cause permanent damage to the heart if left untreated. Antibiotics, such as penicillin, are used in treating the disease.

Rheumatic fever
Classification and external resources
Streptococcus pyogenes bacteria Pappenheim's stain
ICD-10	I00.-I02.
ICD-9	390–392
DiseasesDB	11487
MedlinePlus	003940
eMedicine	med/3435 med/2922 emerg/509 ped/2006
MeSH	D012213

Rheumatic fever is an inflammatory disease that may develop two to three weeks after a Group A streptococcal infection (such as strep throat or scarlet fever). It is believed to be caused by antibody cross-reactivity and can involve the heart, joints, skin, and brain^[1]. Acute rheumatic fever commonly appears in children between ages 5 and 15, with only 20% of first time attacks occurring in adults^[1].

It gets its name for its similarity in presentation to rheumatism.^[2]

Contents 1 General information 2 Diagnosis: modified Jones criteria 2.1 Major criteria 2.2 Minor criteria 2.3 Other signs and symptoms 3 Pathophysiology 4 Treatment 4.1 Infection 4.2 Inflammation 4.3 Heart failure 5 Prevention 6 Notable people affected by rheumatic fever 7 References 8 External links

General information

Rheumatic heart disease at autopsy with characteristic findings (thickened mitral valve, thickened chordae tendineae, hypertrophied left ventricular myocardium).

Rheumatic fever is common worldwide and responsible for many cases of damaged heart valves. In Western countries, it became fairly rare since the 1960s, probably due to widespread use of antibiotics to treat streptococcus infections. While it is far less common in the United States since the beginning of the 20th century, there have been a few outbreaks since the 1980s. Although the disease seldom occurs, it is serious and has a mortality of 2–5%.^[3]

Rheumatic fever primarily affects children between ages 5 and 15 years and occurs approximately 20 days after strep throat or scarlet fever. In up to a third of cases, the underlying strep infection may not have caused any symptoms.

The rate of development of rheumatic fever in individuals with untreated strep infection is estimated to be 3%. The incidence of recurrence with a subsequent untreated infection is substantially greater (about 50%).^[4] The rate of development is far lower in individuals who have received antibiotic treatment. Persons who have suffered a case of rheumatic fever have a tendency to develop flare-ups with repeated strep infections.

The recurrence of rheumatic fever is relatively common in the absence of maintenance of low dose antibiotics, especially during the first three to five years after the first episode. Heart complications may be long-term and severe, particularly if valves are involved.

Survivors of Rheumatic fever often have to take penicillin to prevent streptococcal infection which could possibly lead to another case of Rheumatic fever that could prove fatal.

Diagnosis: modified Jones criteria

T. Duckett Jones, MD, first published these criteria in 1944.^[5] They have been periodically revised by the American Heart Association in collaboration with other groups.^[6] According to revised Jones criteria, the diagnosis of rheumatic fever can be made when two of the major criteria, or one major criterion plus two minor criteria, are present along with evidence of streptococcal infection. Exceptions are chorea and indolent carditis, each of which by itself can indicate rheumatic fever. ^[7][8][9]

Major criteria

• Migratory polyarthritis: a temporary migrating inflammation of the large joints, usually starting in the legs and migrating upwards.
• Carditis: inflammation of the heart muscle which can manifest as congestive heart failure with shortness of breath, pericarditis with a rub, or a new heart murmur.
• Subcutaneous nodules: painless, firm collections of collagen fibers over bones or tendons. They commonly appear on the back of the wrist, the outside elbow, and the front of the knees.
• Erythema marginatum: a long lasting rash that begins on the trunk or arms as macules and spreads outward to form a snake like ring while clearing in the middle. This rash never starts on the face and it is made worse with heat.
• Sydenham's chorea (St. Vitus' dance): a characteristic series of rapid movements without purpose of the face and arms. This can occur very late in the disease.

Minor criteria

• Fever
• Arthralgia: Joint pain without swelling
• Raised Erythrocyte sedimentation rate or C reactive protein
• Leukocytosis
• ECG showing features of heart block, such as a prolonged PR interval^[10]
• Supporting evidence of Streptococcal infection: elevated or rising Antistreptolysin O titre or DNAase.^[1].
• Previous episode of rheumatic fever or inactive heart disease

Other signs and symptoms

• Abdominal pain
• Nose bleeds

Pathophysiology

Rheumatic fever is a systemic disease affecting the peri-arteriolar connective tissue and can occur after an untreated Group A Beta hemolytic streptococcal pharyngeal infection. It is believed to be caused by antibody cross-reactivity. This cross-reactivity is a Type II hypersensitivity reaction and is termed molecular mimicry. Usually, self reactive B cells remain anergic in the periphery without T cell co-stimulation. During a Strep. infection, mature antigen presenting cells such as B cells present the bacterial antigen to CD4-T cells which differentiate into helper T₂ cells. Helper T₂ cells subsequently activate the B cells to become plasma cells and induce the production of antibodies against the cell wall of Streptococcus. However the antibodies may also react against the myocardium and joints^[11], producing the symptoms of rheumatic fever.

Group A streptococcus pyogenes has a cell wall composed of branched polymers which sometimes contain "M proteins" that are highly antigenic. The antibodies which the immune system generates against the "M proteins" may cross react with cardiac myofiber protein myosin^[12],heart muscle glycogen and smooth muscle cells of arteries, inducing cytokine release and tissue destruction. However, the only proven cross reaction is with perivascular connective tissue.^{[citation needed]} This inflammation occurs through direct attachment of complement and Fc receptor-mediated recruitment of neutrophils and macrophages. Characteristic Aschoff bodies, composed of swollen eosinophilic collagen surrounded by lymphocytes and macrophages can be seen on light microscopy. The larger macrophages may become Aschoff giant cells. Acute rheumatic valvular lesions may also involve a cell-mediated immunity reaction as these lesions predominantly contain T-helper cells and macrophages.^[13]

In acute RF, these lesions can be found in any layer of the heart and is hence called pancarditis. The inflammation may cause a serofibrinous pericardial exudates described as “bread-and-butter” pericarditis, which usually resolves without sequelae. Involvement of the endocardium typically results in fibrinoid necrosis and verrucae formation along the lines of closure of the left-sided heart valves. Warty projections arise from the deposition, while subendothelial lesions may induce irregular thickenings called MacCallum plaques.

Chronic rheumatic heart disease is characterized by repeated inflammation with fibrinous resolution. The cardinal anatomic changes of the valve include leaflet thickening, commissural fusion and shortening and thickening of the tendinous cords. ^[13]

Treatment

The management of acute rheumatic fever is geared toward the reduction of inflammation with anti-inflammatory medications such as aspirin or corticosteroids. Individuals with positive cultures for strep throat should also be treated with antibiotics. Aspirin is the drug of choice and should be given at high doses of 100 mg/kg/day. One should watch for side effects like gastritis, salicylate poisoning etc. Steroids are reserved for cases where there is evidence of involvement of heart. The use of steroids may prevent further scarring of tissue and may prevent development of sequelae such as Mitral stenosis. Monthly injections of Longacting Penicillin must be given for a period of 5 years in patients having one attack of Rheumatic fever. If there is evidence of carditis, the length of Penidure therapy may be up to 40 years. Another important cornerstone in treating rheumatic fever includes the continual use of low dose antibiotics (such as penicillin, sulfadiazine, or erythromycin) to prevent recurrence.

Infection

Patients with positive cultures for Streptococcus pyogenes should be treated with penicillin as long as allergy is not present. This treatment will not alter the course of the acute disease.

Inflammation

Patients with significant symptoms may require corticosteroids. Salicylates are useful for pain.

Heart failure

Some patients develop significant carditis which manifests as congestive heart failure. This requires the usual treatment for heart failure: diuretics and digoxin. Unlike normal heart failure, rheumatic heart failure responds well to corticosteroids.

Prevention

Prevention of recurrence is achieved by eradicating the acute infection and prophylaxis with antibiotics. The American Heart Association recommends daily or monthly prophylaxis continue long-term, perhaps for life.^[14]

Screening school-aged children for sore throats also aid in prevention.

Notable people affected by rheumatic fever

This section does not cite any references or sources. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed. (November 2009)

• Alois Alzheimer (likely though not certain)
• Bobby Bowden
• Jeremy Brett
• Robert Burns
• Lou Costello
• Bobby Darin
• Billy Fury
• Kurt Gödel
• Franz Kline
• Archibald Lampman
• William Pitt Leleiohoku
• Carson McCullers
• Wolfgang Amadeus Mozart (likely though not certain)
• Michael O'Donoghue
• Charles Stewart Parnell (likely though not certain)
• Richard Proenneke
• Massimo Troisi
• Princess Astrid of Norway

References

1. ^ ^{a b c} Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; & Mitchell, Richard N. (2007). Robbins Basic Pathology (8th ed.). Saunders Elsevier. pp. 403-406 ISBN 978-1-4160-2973-1
2. ^ rheumatic fever at Dorland's Medical Dictionary
3. ^ Medline Plus Medical Encyclopedia: Rheumatic fever
4. ^ Porth, Carol (2007). Essentials of pathophysiology: concepts of altered health states. Hagerstown, MD: Lippincott Williams & Wilkins. ISBN 0-7817-7087-4. 
5. ^ Jones TD (1944). "The diagnosis of rheumatic fever.". JAMA 126: 481–4. 
6. ^ Ferrieri P (2002). "Proceedings of the Jones Criteria workshop". Circulation 106 (19): 2521–3. doi:10.1161/01.CIR.0000037745.65929.FA. PMID 12417554. http://circ.ahajournals.org/cgi/content/full/106/19/2521?ck=nck. 
7. ^ Steven J Parrillo, DO, FACOEP, FACEP. "eMedicine — Rheumatic Fever". http://www.emedicine.com/emerg/topic509.htm. Retrieved 2007-07-14. 
8. ^ "Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young of the American Heart Association". JAMA 268 (15): 2069–73. 1992. PMID 1404745. 
9. ^ Saxena, Anita (2000). "Diagnosis of rheumatic fever: Current status of Jones criteria and role of echocardiography". Indian Journal of Pediatrics 67 (4): 283–6. doi:10.1007/BF02758174. PMID 11129913. 
10. ^ http://www.utmb.edu/Pedi_Ed/CoreConceptsOfPediatrics/Cardiology/page_40.htm
11. ^ Abbas and Lechtman. Basic Immunology: Functions and Disorders of the Immune System. Elsevier Inc. 2004.
12. ^ Faé KC, da Silva DD, Oshiro SE, et al. (May 2006). "Mimicry in recognition of cardiac myosin peptides by heart-intralesional T cell clones from rheumatic heart disease". J. Immunol. 176 (9): 5662–70. PMID 16622036. http://www.jimmunol.org/cgi/pmidlookup?view=long&pmid=16622036. 
13. ^ ^{a b} Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease. St. Louis, Mo: Elsevier Saunders. ISBN 0-7216-0187-1. http://www.robbinspathology.com/. 
14. ^ "Rheumatic Heart Disease/Rheumatic Fever". American Heart Association. http://www.americanheart.org/presenter.jhtml?identifier=4709. Retrieved 2008-02-17. 

External links

• Rheumatic fever information from Seattle Children's Hospital Heart Center
• Jones major criteria, Mnemonic

v • d • e Infectious diseases · Bacterial diseases: G+ (primarily A00–A79, 001–041, 080–109) Firmicutes/ (low-G+C) Bacilli Lactobacillales (Cat-) Streptococcus Alpha hemolytic optochin susceptible: S. pneumoniae (Pneumococcal infection) optochin resistant: S. viridans (S. mitis, S. mutans, S. oralis, S. sanguinis, S. sobrinus, milleri group) Beta hemolytic A, bacitracin susceptible: S. pyogenes (Scarlet fever, Erysipelas, Rheumatic fever, Streptococcal pharyngitis) B, bacitracin resistant, CAMP test+: S. agalactiae Gamma hemolytic D, BEA+: Streptococcus bovis Enterococcus BEA+: Enterococcus faecalis (Urinary tract infection) · Enterococcus faecium Bacillales (Cat+) Staphylococcus Cg+ S. aureus (Staphylococcal scalded skin syndrome, Toxic shock syndrome, MRSA) Cg- novobiocin susceptible (S. epidermidis) · novobiocin resistant (S. saprophyticus) Bacillus Bacillus anthracis (Anthrax) · Bacillus cereus (Food poisoning) Listeria Listeria monocytogenes (Listeriosis) Clostridia Clostridium (spore-forming) motile: Clostridium difficile (Pseudomembranous colitis) · Clostridium botulinum (Botulism) · Clostridium tetani (Tetanus) nonmotile: Clostridium perfringens (Gas gangrene, Clostridial necrotizing enteritis) Peptostreptococcus (non-spore forming) Peptostreptococcus magnus Mollicutes Mycoplasmataceae Ureaplasma urealyticum (Ureaplasma infection) · Mycoplasma genitalium · Mycoplasma pneumoniae (Mycoplasma pneumonia) Anaeroplasmatales Erysipelothrix rhusiopathiae (Erysipeloid) Actinobacteria/ (high-G+C) Actinomycineae Actinomycetaceae Actinomyces israelii (Actinomycosis) · Tropheryma whipplei (Whipple's disease) Propionibacteriaceae Propionibacterium acnes Corynebacterineae Mycobacteriaceae M. tuberculosis/M. bovis Tuberculosis: Ghon focus/Ghon's complex · Pott disease · brain (Meningitis, Rich focus) · cutaneous (Scrofula, Bazin disease, Lupus vulgaris, Prosector's wart) · Miliary · Tuberculous pericarditis M. leprae Leprosy Nontuberculous R1: M. kansasii · M. marinum R2: M. gordonae R3: M. avium complex (MAA, MAP, MAI, Lady Windermere syndrome) · M. ulcerans (Buruli ulcer) · M. haemophilum R4: M. fortuitum · M. chelonae Nocardiaceae Nocardia asteroides/Nocardia brasiliensis (Nocardiosis) · Rhodococcus equi Corynebacteriaceae Corynebacterium diphtheriae (Diphtheria) · Corynebacterium minutissimum (Erythrasma) · Corynebacterium jeikeium Bifidobacteriaceae Gardnerella vaginalis

v • d • e Cardiovascular disease: heart disease · Circulatory system pathology (I00-I52, 390-429) Ischaemic CD/CHD CAD · Coronary thrombosis · Coronary vasospasm · Coronary artery aneurysm · Myocardial Bridge Active ischemia Angina pectoris (Prinzmetal's angina, Stable angina) · Acute coronary (Unstable angina, Myocardial infarction / heart attack) Sequelae hours (Myocardial stunning, Hibernating myocardium) · days (Myocardial rupture) · weeks (Aneurysm of heart/Ventricular aneurysm, Dressler's syndrome) Layers Pericardium Pericarditis (Acute, Chronic/Constrictive) · Pericardial effusion (Hemopericardium, Cardiac tamponade) Myocardium Myocarditis (Chagas disease) Cardiomyopathy: Dilated (Alcoholic) · Hypertrophic · Restrictive (Loeffler endocarditis, Cardiac amyloidosis, Endocardial fibroelastosis) Arrhythmogenic right ventricular dysplasia Endocardium/ valves Endocarditis Infective endocarditis (Subacute bacterial endocarditis) · noninfective endocarditis (Nonbacterial thrombotic endocarditis, Libman-Sacks endocarditis) Valves mitral (regurgitation, prolapse, stenosis) · aortic (stenosis, insufficiency) · tricuspid (stenosis, insufficiency) · pulmonary (stenosis, insufficiency) Conduction/ arrhythmia Bradycardia Sinus bradycardia · Sick sinus syndrome Heart block: Sinoatrial · AV (1°, 2°, 3°) · Intraventricular (Bundle branch/Right/Left, Left anterior fascicular/Left posterior fascicular, Bifascicular/Trifascicular) · Adams-Stokes syndrome Tachycardia (paroxysmal and sinus) Supraventricular Atrial (Multifocal) · Junctional (AV nodal reentrant, Junctional ectopic) Ventricular Torsades de pointes · Catecholaminergic polymorphic · Accelerated idioventricular rhythm Premature contraction Atrial · Ventricular Pre-excitation syndrome Wolff-Parkinson-White · Lown-Ganong-Levine Flutter/fibrillation Atrial flutter · Ventricular flutter · Atrial fibrillation (Familial) · Ventricular fibrillation Pacemaker Wandering pacemaker · Ectopic pacemaker/Ectopic beat · Parasystole · Multifocal atrial tachycardia · Pacemaker syndrome Long QT syndrome Romano-Ward syndrome · Andersen-Tawil syndrome · Jervell and Lange-Nielsen syndrome Cardiac arrest Sudden cardiac death · Asystole · Pulseless electrical activity · Sinoatrial arrest Other/ungrouped hexaxial reference system (Right axis deviation, Left axis deviation) · QT (Short QT syndrome) · T (T wave alternans) · ST (Osborn wave, ST elevation, ST depression) Cardiomegaly Ventricular hypertrophy (Left, Right/Cor pulmonale) · Atrial enlargement (Left, Right) Other Cardiac fibrosis · Heart failure (Cardiac asthma) · Rheumatic fever heart navs: anat/physio/dev, noncongen/congen/neoplasia, symptoms+signs/eponymous, proc

v • d • e Immune disorders: hypersensitivity and autoimmune diseases Type I/allergy/atopy (IgE) Foreign Atopic dermatitis · Allergic urticaria · Hay fever · Allergic asthma · Anaphylaxis · Food allergy (Milk, Egg, Peanut, Tree nut, Seafood, Soy, Wheat), Penicillin allergy Autoimmune none Type II/ADCC (IgM, IgG) Foreign Pernicious anemia · Hemolytic disease of the newborn Autoimmune Cytotoxic Autoimmune hemolytic anemia · Idiopathic thrombocytopenic purpura  · Bullous pemphigoid  · Pemphigus vulgaris · Rheumatic fever · Goodpasture's syndrome "Type 5"/receptor Graves' disease · Myasthenia gravis Type III (Immune complex) Foreign Henoch-Schönlein purpura · Hypersensitivity vasculitis · Reactive arthritis · Rheumatoid arthritis · Farmer's lung · Post-streptococcal glomerulonephritis · Serum sickness · Arthus reaction Autoimmune Systemic lupus erythematosus · Subacute bacterial endocarditis Type IV/cell-mediated (T-cells) Foreign Contact dermatitis · Mantoux test Autoimmune Diabetes mellitus type 1 · Hashimoto's thyroiditis · Guillain-Barré syndrome  · Multiple sclerosis  · Coeliac disease · Giant cell arteritis GVHD Transfusion-associated graft versus host disease Unknown/ multiple Foreign Hypersensitivity pneumonitis (Allergic bronchopulmonary aspergillosis) · Transplant rejection · Latex allergy (I+IV) Autoimmune Sjögren's syndrome · Autoimmune hepatitis · Autoimmune polyendocrine syndrome (APS1, APS2) · Autoimmune adrenalitis lymphocyte navs: cells/physio, immunodeficiency/immunoproliferative immunoglobulin/neoplasia, proc

This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see full disclaimer)