rheumatic fever

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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

 [roo-mat´ik]
pertaining to or affected with rheumatism.
rheumatic fever a disease associated with the presence of hemolytic streptococci in the body. It is called rheumatic fever because two common symptoms are fever and pain in the joints similar to that of rheumatism. It is relatively common, particularly in children between 5 and 15 years old; young adults in the early twenties are also susceptible.
Causes. Rheumatic fever is a delayed sequela of an upper respiratory infection caused by the Group A hemolytic streptococcus that causes such common childhood illnesses as scarlet fever, tonsillitis, streptococcal sore throat (“strep throat”), and ear infections. It is only one of several complications that can result from a streptococcal infection.



The connection between rheumatic fever and a previous streptococcal infection has been proved only indirectly. In almost all cases of rheumatic fever there is evidence of previous streptococcal infection, and when the infection is treated promptly, the likelihood of rheumatic fever decreases sharply. There is evidence that the symptoms of rheumatic fever may result from an antigen-antibody reaction to one or more of the products of the hemolytic streptococcus, but the exact way this happens is not known. Rheumatic fever has been classified as an autoimmune disease. It tends to run in families, indicating a possible hereditary predisposition. Economic and environmental conditions such as a damp, cold climate and poor health habits may also be contributing factors.
Symptoms. The initial symptoms usually appear 1 to 4 weeks after the streptococcal infection has occurred. The actual onset of the disease may be either gradual or sudden. The symptoms vary widely and may be of any degree of severity. The most common initial complaints are a slight fever, a feeling of tiredness, a vague feeling of pain in the limbs, and nosebleeds. If the disease takes an acute form, the fever may reach 40°C (104°F) by the second day and continue for several weeks, although the usual course of the fever is about 2 weeks. On the other hand, the fever may be quite mild.



Joint pain develops at any stage of the disease and lasts from a few hours to several weeks. The joints swell and are tender to the touch. The pain and swelling often subside in one group of joints and arise in another. As the pain subsides, the joints return to normal.

Other symptoms may include the spasmodic twitching movements known as sydenham's chorea, especially in girls between the ages of 6 and 11. A rash caused by the fever may appear upon the body. Nodules may be seen or felt under the skin at the elbow, knee, and wrist joints, and along the spine. Among the most serious signs is the development of a heart murmur and cardiac decompensation.
Heart Damage. The seriousness of rheumatic fever lies primarily in the permanent damage it can do to the heart. The disease tends to recur, and the recurrent attacks may further weaken the heart. The usual cardiac complication is endocarditis (inflammation of the inner lining of the heart, including the membrane over the valves). As a valve heals, its edges may become so scarred and stiff that they fail to close properly. As a result, blood leaks through the valve when it is closed, producing the sound characteristic of a heart murmur. The valves may become thickened with scar tissue, so that the amount of blood that can flow through the heart is restricted. If there is severe stenosis of the mitral valve and the patient develops symptoms of congestive heart failure, surgery to enlarge the valve (mitral commissurotomy) may be indicated.
Treatment. The main purposes of treatment are reduction of fever and pain and promotion of the natural healing processes; no means have yet been discovered for fighting the disease directly. Until the introduction of antibiotics and steroids, the chief medications were aspirin and other salicylates. Penicillin is prescribed if there is evidence of an ongoing streptococcal infection or the chance of exposure to streptococcal infection. Prednisone may be prescribed to reduce the pain and swelling in the joints, but its effect on the ultimate course of the disease is controversial. If pain is severe, analgesic drugs may be given. Bed rest is an important part of the treatment, particularly if the disease has caused heart damage. Depending upon the severity of the disease, the patient may be kept in bed for months, and prolonged convalescence may be needed.
Patient Care. In the acute phase of rheumatic fever rest is most important to reduce the work load of the heart. The patient should be made as comfortable as possible and disturbed only when necessary. The care should be planned so that long periods of complete rest are possible. Proper positioning with adequate support of the limbs and maintenance of good body alignment is essential to rest and the prevention of complications.



The temperature, pulse, and respirations are checked and recorded at least every 4 hours during the day. The volume and rhythm as well as the rate of the pulse should be noted. The blood pressure is taken at least once a day. Fluid intake may be restricted if there is edema, and sodium intake may also be limited; in either case the reason for the restriction should be explained to the patient. A record is kept of the intake and output.

Frequent back care and good oral hygiene are needed to promote comfort and relaxation. When turning the patient, one should be gentle and slow, avoiding unnecessary handling of the joints, which may be tender and swollen.

During the convalescent period the patient is allowed a gradual return to physical activities. The amount of activity depends on the physician's orders and is based on the patient's pulse rate, erythrocyte sedimentation rate, and C-reaction protein test. Measures must be taken to avoid respiratory infections, which will retard the progress of the patient. Small, frequent feedings that provide a well-balanced diet are usually preferred to three meals a day, which may be only partially eaten by a patient who is not engaging in a normal amount of physical activity.

As the need for rest is decreased, some provision must be made for diversional activities that will help eliminate boredom and keep the child content. The psychologic effects of a prolonged period of enforced dependence on others must also be considered. The parents and the child will need encouragement and help in the transition from total dependence to relative independence. Parents and family members also will need support and guidance during adjustment to home care of the child. Referral to the public health nurse or home health care agency can help provide continuity of care and continued support.
Prevention. Preventive care is extremely important, especially when rheumatic fever has once occurred, since it tends to return unless precautionary steps are taken. The patient is given penicillin, orally every day or by intramuscular injection once a month, for many years in order to prevent streptococcal infection. A good nutritious diet and sufficient sleep are important. Administration of antibiotics to all patients with history of rheumatic fever undergoing even minor surgery, including tooth extraction, is important in preventing bacterial endocarditis. Prompt and effective treatment of “strep throat” among the general population has reduced the incidence of rheumatic fever.
Pathogenesis of rheumatic fever. Following infection (“strep throat”), an immune response elicited by the streptococci acts on the heart and several other organs, most notably the joints, skin, and central nervous system. In the heart, it causes endocarditis, myocarditis, and pericarditis. From Damjanov, 2000.
Manifestations of rheumatic fever. From Betz et al., 1994.
rheumatic heart disease the most important and constant manifestation of rheumatic fever, consisting of inflammatory changes with valvular deformities.

rheu·mat·ic fe·ver

a subacute febrile syndrome occurring after group A β-hemolytic streptococcal infection (usually pharyngitis) and mediated by an immune response to the organism; most often seen in children and young adults; features include fever, myocarditis (causing tachycardia and sometimes acute cardiac failure), endocarditis (with valvular incompetence, followed after healing by scarring), and migratory polyarthritis; less often, subcutaneous nodules, erythema marginatum, and Sydenham chorea; relapses can occur after reinfection with streptococci. See: Jones criteria.

rheumatic fever

n.
An acute inflammatory disease occurring after a streptococcal infection such as strep throat, characterized by fever and joint pain and often resulting in permanent heart damage.

rheumatic fever (rf)

a systemic inflammatory disease that may develop as a delayed reaction to an inadequately treated infection of the upper respiratory tract by group A beta-hemolytic streptococci. The disease usually occurs in young school-age children and may affect the brain, heart, joints, skin, or subcutaneous tissues. Also called acute articular rheumatism. See also rheumatic heart disease.
observations The onset of rheumatic fever is usually sudden, often occurring 1 to 5 symptom-free weeks after recovery from a sore throat or scarlet fever. Early symptoms generally include fever, joint pain, nosebleeds, abdominal pain, and vomiting. The major manifestations of this disease include migratory polyarthritis affecting numerous joints and carditis, which causes palpitations, chest pain, and, in severe cases, symptoms of cardiac failure. Sydenham's chorea is usually the sole late sign of rheumatic fever and may initially be manifested as an increased awkwardness and tendency to drop objects. As the chorea progresses, irregular body movements may become extensive, occasionally involving the tongue and facial muscles, resulting in incapacitation. Other developments may include transient erythema marginatum with circular lesions and subcutaneous rheumatic nodules on various joints and tendons, the spine, and the back of the head. There is no specific diagnostic test for rheumatic fever. The development of serum antibodies to streptococcal antigens is a positive diagnostic sign. Affected individuals may also develop leukocytosis, moderate anemia, and proteinuria. C-reactive protein, evaluated in a specimen of blood, is abnormally high in concentration. Recurrences of rheumatic fever are common. Except for carditis, all the manifestations of the disease usually subside without any permanent effects. Mild cases may last 3 to 4 weeks. Severe cases with associated arthritis and carditis may last 2 to 3 months.
interventions Management of rheumatic fever includes bed rest and severe restriction of normal activity. Penicillin is often administered, even if throat cultures are negative, and steroids or salicylates may be used, depending on the severity of any associated carditis and arthritis.
nursing considerations Symptoms largely determine the type of nursing care. The nurse is alert to signs of toxicity associated with salicylate, steroid, and antibiotic therapies. The nurse also monitors the patient's fluid status with regard to cardiac function, helps minimize joint pains by properly positioning the patient, and gives emotional support.

rheumatic fever

Rheumatology The late non-purulent sequelae of a URI by streptococcus group A Diagnosis Major criteria–carditis, chorea, erythema marginatum, polyarthritis, subcutaneous nodules; minor criteria–arthralgia, fever, Hx of previous rheumatic fever, or evidence of cardiac involvement, lab parameters ↑ acute phase reactants, anti-streptolysin O titers, C-reactive protein, ESR, delineated by Jones and later modified. See 'Chinese menu disease, ' Jones criteria.

rheu·mat·ic fe·ver

(rū-mat'ik fē'vĕr)
An inflammatory disease with pyrexia following infection of the throat with group A beta-hemolytic streptococci, occurring primarily in children and young adults, and inducing an immunopathy variably associated with acute migratory polyarthritis, Sydenham chorea, subcutaneous nodules over bony prominences, myocarditis with formation of Aschoff bodies that may cause acute cardiac failure, and endocarditis (frequently followed by scarring of valves, causing stenosis or incompetence); relapses are common if streptococcal infections recur.

rheumatic fever

A disease, now rare in the Western world but still endemic in developing countries, caused by an ANTIGEN present on certain strains of STREPTOCOCCI. This is closely similar to an antigen on the muscle fibres of the heart and elsewhere. Antibodies are produced by the body and these attack the heart and other parts. Joint involvement is fleeting and unimportant but damage to the heart valves and sometimes the nervous system is permanent. Possible sequels are heart valve leakage (incompetence) or narrowing (stenosis) and ‘St Vitus’ dance’ (SYDENHAM'S CHOREA). Rheumatic fever and subsequent damage can be avoided by treating streptococcal infections with penicillin. Sydenham's chorea is helped by tranquillizer drugs and sedatives.

rheumatic fever

pyrexia subsequent to group A streptococcal throat infection that predisposes to scarring of heart valves and cardiac incompetence

rheu·mat·ic fe·ver

(rū-mat'ik fē'vĕr)
An inflammatory disease with pyrexia following infection of the throat with group A beta-hemolytic streptococci, occurring primarily in children and young adults.
References in periodicals archive ?
Acute rheumatic fever in Auckland, New Zealand: spectrum of associated group A streptococci different from expected.
Aydin and his coinvestigators have also studied polymorphisms in TLR-4 but saw no association with acute rheumatic fever.
The worldwide epidemiology of acute rheumatic fever and rheumatic heart disease.
Table 1 Diagnosing Acute Rheumatic Fever, "Jones Criteria," 1992 Update Major Carditis, polyarthritis, chorea, erythema manifestations marginatum, subcutaneous nodules Minor Arthralgias, fever, elevated erythrocyte manifestations sedimentation rate, elevated C-reactive protein, prolonged PR interval on EKG Evidence of Recent past positive throat culture or rapid antecedent antigen test, elevated or rising group A strep anti-streptolysin 0, or anti-DNase B infection antibody titers.
Carditis is a rare presentation of acute rheumatic fever in an adult.
Although strep throat can be diagnosed by means of a throat culture, there is no single symptom, sign or laboratory test that is diagnostic of acute rheumatic fever.
Dale stated "The worldwide prevalence of streptococcal infections and acute rheumatic fever and the recent resurgence of deadly invasive group A streptococcal infections and rheumatic fever make the development of a safe and effective vaccine an urgent medical issue.
A marked decline in children presenting with acute rheumatic fever (ARF) and chronic rheumatic heart disease (RHD) over the past two decades has been observed at Chris Hani Baragwanath Academic Hospital in Soweto.
Acute rheumatic carditis is an important healthcare problem in developing countries and continues to be the leading cause of acquired heart disease in children worldwide, and acute rheumatic fever (ARF) occurs as a complication of an untreated group A [beta]-hemolytic streptococcal pharyngitis (4, 5).
A new report has revealed that young Maori are 23 times more likely to suffer acute rheumatic fever than non-Maori.

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