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Acute Rheumatic Fever

Cause

• ARF is believed to be an immunologic lesion that
occurs as a delayed sequela of group A
streptococcal infection of the pharynx but not of
the skin. The attack rate of ARF after streptococcal
infection varies with the severity of the infection,
ranging from 0.3% to 3%.

• Important predisposing factors include family history
of rheumatic fever, low socioeconomic status
(poverty, poor hygiene, medical deprivation), and age
between 6 and 15 years (with a peak incidence at 8
years of age).

Pathology  uncleare

• The inflammatory lesion is found in many parts of the
body, most notably in the heart, brain, joints, and
skin. Valvular damage most frequently involves the
mitral, less commonly the aortic, and rarely the
tricuspid and pulmonary valves.

• Aschoff bodies in the atrial myocardium are believed
to be characteristic of rheumatic fever. These
consist of inflammatory lesions associated with
swelling, fragmentation of collagen fibers, and
alterations in the staining characteristics of
connective tissue.

Clinical Manifestations • ARF is diagnosed by the use of revised Jones criteria • The criteria are three groups of important clinical and laboratory findings: (1) five major manifestations (2) four minor manifestations (3) supporting evidence of an antecedent group A streptococcal infection. .

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• • Pallor. The latent period may be as long as 2 to 6 months (average. is common.HISTORY • History of streptococcal pharyngitis. 3 weeks) before the onset of symptoms. easy fatigability. 1 to 5 weeks (average. may be present. 4 months) in cases of isolated chorea. . and other history. such as epistaxis (5% to 10%) and abdominal pain. malaise. • Family history of rheumatic fever frequently is positive.

is involved • characteristic migratory nature of the arthritis.. Often more than one joint. • The arthritis responds dramatically to salicylate therapy. .MAJOR MANIFESTATIONS Arthritis • Arthritis. and limitation of motion are common. wrists). if patients treated with salicylates (with documented therapeutic levels) do not improve in 48 hours. either simultaneously or in succession. redness. Swelling. the relationship of this disease to acute rheumatic fever remains undetermined. knees. the most common manifestation of ARF (70% of cases) • involves large joints (e. ankles. the diagnosis of ARF probably is incorrect.g. tenderness. severe pain. • Such patients have been categorized as having “poststreptococcal reactive arthritis”. heat. elbows.

• Pericarditis (friction rub. carditis should not be diagnosed. chest pain. without the murmurs of MR and/or AR. without accompanying auscultatory findings. cardiomegaly) are indications of severe carditis. pancarditis. its absence makes the diagnosis of myocarditis unlikely. distant heart sounds.Carditis Carditis occurs in 50% of patients: • Tachycardia (out of proportion to the degree of fever) is common. • the presence of MR or AR by echo and Doppler studies. cannot be taken as the sole criterion for valvulitis. • Signs of CHF (gallop rhythm. • A heart murmur of valvulitis (caused by mitral regurgitation [MR] and/or aortic regurgitation [AR]) is almost always present. and ECG changes) may be present. . or congestive heart failure (CHF). • Cardiomegaly on chest x-ray films is indicative of pericarditis. pericardial effusion.

Erythema Marginatum • Occurs in <10% of patients with ARF. . disappearing on exposure to cold and reappearing after a hot shower or when the patient is covered with a warm blanket. They seldom are detected in air-conditioned hospital rooms. they are never seen on the face. • The rashes are evanescent. • The characteristic nonpruritic serpiginous or annular erythematous rashes are most prominent on the trunk and the inner proximal portions of the extremities.

Not transient. freely movable. singly or in clusters.Subcutaneous Nodules Found in 2% to 10% of patients. particularly in those with recurrences. Hard. over the scalp. lasting for weeks. on the extensor surfaces of both large and small joints. and have a significant association with carditis. or along the spine. swelling. Symmetris. painless.2 to 2 cm in diameter. nonpruritic. and 0. .

• spontaneous. obsessions and compulsions). Vitus' dance) is found in 15% of patients with ARF. hyperactivity. . emotional lability. • more often in prepubertal girls (8 to 12 years) than in boys. • neuropsychiatric disorder consisting of both neurologic signs (choreic movement and hypotonia) and psychiatric signs (e.g. • begins with emotional lability and personality changes.. purposeless movement of chorea (which lasts 4 to 18 months). separation anxiety. followed by motor weakness.Sydenham's Chorea • Sydenham's chorea (St.

. weakness. and hypotonia continue for an average of 7 months (up to 17 months) before slowly waning in severity. • These findings suggest that chorea may be related to dysfunction of basal ganglia and cortical neuronal components. elevated titers of “antineuronal antibodies” recognizing basal ganglion tissues have been found in over 90% of patients. Sydenham's Chorea… • The distractability and inattentiveness outlast the choreic movements. • Recently. The levels of the antineuronal antibody titer are positively related to the severity of choreic movements. • The adventitious movements.

• Fever (usually with a temperature of at least 102°F [38.8°C]) is present early in the course of untreated rheumatic fever. .MINOR MANIFESTATIONS • Arthralgia refers to joint pain without the objective changes of arthritis. elevated acute-phase reactants (elevated C- reactive protein levels and elevated erythrocyte sedimentation rate) are objective evidence of an inflammatory process. • A prolonged PR interval on the ECG is neither specific for ARF nor an indication of active carditis. • In laboratory findings. It must not be considered a minor manifestation when arthritis is present.

• Streptococcal antibody tests are the most reliable laboratory evidence of antecedent streptococcal infection capable of producing ARF. The onset of the clinical manifestations of ARF coincides with the peak of the streptococcal antibody response. . a negative test result should be confirmed by a conventional throat culture. Antigen detection tests are very specific but not very sensitive. • Positive throat cultures or rapid streptococcal antigen tests for group A streptococci are less reliable than antibody tests because they do not distinguish between recent infection and chronic pharyngeal carriage.EVIDENCE OF ANTECEDENT GROUP A STREPTOCOCCAL INFECTION • A history of sore throat or of scarlet fever unsubstantiated by laboratory data is not adequate evidence of recent group A streptococcal infection.

– Only 67% of patients with isolated chorea have an elevated ASO titer. If three other antistreptococcal antibody tests (antideoxyribonuclease B. Titers of 240 Todd units or greater in children and 120 Todd units or greater in adults are considered elevated. – The antideoxyribonuclease B test is favored over other tests. ASO titers of at least 333 Todd units in children and 250 Todd units in adults are considered elevated. . It is elevated in 80% of patients with ARF and in 20% of normal individuals. antistreptokinase. – The Streptozyme test (Wampole Laboratories) is a relatively simple slide agglutination test.– Antistreptolysin O (ASO) titer is well standardized and therefore is the most widely used test. – A single low ASO titer does not exclude ARF. but it is less standardized and less reproducible than the other antibody tests. and antihyaluronidase tests) are obtained. a titer for at least one antibody test is elevated in about 95% of patients.

and precordial pain are relatively common but not specific. rapid sleeping heart rate. anemia. • A positive family history of rheumatic fever also may heighten the suspicion but cannot be used as a diagnostic manifestation. tachycardia out of proportion to fever.OTHER CLINICAL FEATURES • Abdominal pain. malaise. . epistaxis.

• The absence of supporting evidence of a previous group A streptococcal infection makes the diagnosis doubtful. • Exceptions to the Jones criteria include the following three specific situations: – Chorea may occur as the only manifestation of rheumatic fever. are present . patients with rheumatic fever recurrences may not fulfill the Jones criteria. • Only the major and minor criteria and evidence of an antecedent group A streptococcal infection are included in the criteria.Diagnosis • The revised Jones criteria are used for the diagnosis of ARF. plus evidence of antecedent streptococcal infection. – Occasionally. • A diagnosis of ARF is highly probable when either two major manifestations or one major and two minor manifestations. although other findings play a supporting role. . – Indolent carditis may be the only manifestation in patients who come to medical attention months after the onset of rheumatic fever.

respectively. – Arthralgia or a prolonged PR interval cannot be used as a minor manifestation in the presence of arthritis or carditis. – The absence of evidence of an antecedent group A streptococcal infection is a warning that ARF is unlikely (except when chorea is present).. – The possibility of the early suppression of full clinical manifestations should be sought during the history taking.g. Bufferin. Subtherapeutic doses of aspirin or salicylate-containing analgesics (e. – The vibratory innocent (Still's) murmur is often misinterpreted as a murmur of MR and thereby is a frequent cause of misdiagnosis (or overdiagnosis) of ARF. Anacin) may suppress full manifestations. The murmur of MR is a regurgitant-type systolic murmur (starting with the S1). A cardiology consultation during the acute phase minimizes the frequency of misdiagnosis. but the innocent murmur is low pitched and an ejection type. .Tips help in applying the Jones criteria: – Two major manifestations are always stronger than one major plus two minor manifestations.

including poststreptococcal arthritis. • Virus-associated acute arthritis (rubella. a more indolent course. herpesvi ruses. and infectious arthritis (such as gonococcal) occasionally require differentiation.Differential Diagnosis • Juvenile rheumatoid arthritis (JRA) is often misdiagnosed as acute rheumatic fever. reactive arthritis. • Other collagen vascular diseases (systemic lupus erythematosus. symmetrical involvement of large joints without migratory arthritis. The following findings suggest JRA rather than ARF: involvement of peripheral small joints. . serum sickness. enteroviruses) is much more common in adults. no evidence of preceding streptococcal infection. pallor of the involved joints. mixed connective tissue disease). parvovirus. should be considered in the differential diagnosis. and the absence of prompt response to salicylate therapy within 24 to 48 hours. • Hematologic disorders. such as sicklemia and leukemia. hepatitis B virus.

and ECG. acute-phase reactants (ESR and CRP).Labaratory • When ARF is suggested by history and physical examination. two-dimensional echo and Doppler studies are usually performed at that time. one should obtain the following laboratory studies: complete blood count. chest x-ray films. throat culture. . ASO titer. • Cardiology consultation is indicated to clarify whether there is cardiac involvement.

• Anti-inflammatory or suppressive therapy with salicylates or steroids must not be started until a definite diagnosis is made. • When the diagnosis of ARF is confirmed. the need for prophylaxis against infective endocarditis also should be emphasized. Early suppressive therapy may interfere with a definite diagnosis of ARF by suppressing full development of joint manifestations and suppressing acute-phase reactants. . one must educate the patient and parents about the need to prevent subsequent streptococcal infection through continuous antibiotic prophylaxis. In patients with cardiac involvement.

.Management: • Bed rest of varying duration is recommended. • Bed rest is followed by a period of indoor ambulation of varying duration before the child is allowed to return to school. • The duration depends on the type and severity of the manifestations and may range from a week (for isolated arthritis) to several weeks for severe carditis.

• The erythrocyte sedimentation rate is a helpful guide to the rheumatic activity and therefore to the duration of restriction of activities. Full activity is allowed when the erythrocyte sedimentation rate has returned to normal. except in children with significant cardiac involvement. .

may be substituted for penicillin. 50 mg/kg per day in two to four doses for 10 days. 0.• Benzathine penicillin G. This serves as the first dose of penicillin prophylaxis as well. In patients allergic to penicillin. erythromycin. .2 million (>30kg) IU/IM.6 (<30kg) to 1. is given to eradicate streptococci.

the therapy is with drawn gradually over 4 to 6 weeks while monitoring acute-phase reactants with dose 75 mg/kg per day • For arthritis. Rapid resolution of joint symptoms with aspirin within 24 to 36 hours is supportive evidence of the arthritis of ARF. aspirin alone is recommended in a dose of 100 mg/kg per day in four to six divided doses. • • For mild to moderate carditis. aspirin therapy is continued for 2 weeks and gradually withdrawn over the following 2 to 6 weeks. . An adequate blood level of salicylates is 20 to 25 mg/100 mL. This dose is continued for 4 to 8 weeks. depending on the clinical response.• Therapy with anti-inflammatory agents should be started as soon as ARF has been diagnosed. After improvement. • Prednisone (2 mg/kg per day in four divided doses for 2 to 6 weeks) is indicated only in cases of severe carditis.

because certain patients with rheumatic carditis are supersensitive to digitalis). beginning with half the usual recommended dose. cool oxygen. .06 mg/kg • Furosemide. • Restriction of sodium and fluid intake.04-0.2 mg/kg. at 4-hour intervals for severe CHF with respiratory distress. • Digoxin (used with caution.  0. 0. • Morphine sulfate. • Prednisone for severe carditis of recent onset. 1 mg/kg every 6 to 12 hours.Treatment of CHF includes some or all of the following: • Complete bed rest with orthopneic position and moist. if indicated.

. – Anti-inflammatory agents are not needed in patients with isolated chorea.5 mg and increasing every 8 hours to 2 g). or steroids. about 25% of patients with isolated chorea (without carditis) develop rheumatic valvular heart disease in 20- year follow-up. – Plasma exchange (to remove antineuronal antibodies) and IV immune globulin therapy (to inactivate the effects of the antineuronal antibodies) are in the experimental stages (by the National Institutes of Health). haloperidol (starting at 0. just as in patients with other rheumatic manifestations.2 million units. – Give benzathine penicillin G. diazepam (Valium). but the preliminary results are promising in reducing the duration of chorea. valproic acid. chlorpromazine (Thorazine). any of the following drugs may be used: phenobarbital (15 to 30 mg every 6 to 8 hours). 1. Without the prophylaxis.Management of Sydenham's chorea: – Reduce physical and emotional stress and use protective measures as indicated. initially for eradication of streptococcus and also every 28 days for prevention of recurrence. – For severe cases.

. • Recurrence of rheumatic fever: The severity of valvular involvement increases with each recurrence.Prognosis The presence or absence of permanent cardiac damage determines the prognosis. The development of residual heart disease is influenced by the following three factors: • Cardiac status at the start of treatment: The more severe the cardiac involvement at the time the patient is first seen. • Regression of heart disease: Evidence of cardiac involvement at the first attack may disappear in 10% to 25% of patients 10 years after the initial attack. Valvular disease resolves more frequently when prophylaxis is followed. the greater the incidence of residual heart disease.

the prophylaxis should be continued longer. must receive prophylaxis. . provided the patient does not have evidence of valvular involvement and is not in a high-risk occupation (e. physicians.. • If the patient has rheumatic valvular disease.g. schoolteachers. nurses). possibly for life. patients should receive prophylaxis indefinitely. The chance of recurrence is highest in the first 5 years after the ARF.Prevention POPULATION • Patients with documented histories of rheumatic fever. DURATION • Ideally. including those with isolated chorea and those without evidence of rheumatic heart disease. • Many cardiologists recommend discontinuing the prophylaxis at age 21 to 25 years.

although not as effective. twice daily. . 250 mg. Alternative methods. 1. • Oral sulfadiazine.METHODS The method of choice for secondary prevention is benzathine penicillin G. are the following: • Oral penicillin V.2 million units given intramuscularly every 28 days (not once a month). twice daily. 1 g once daily (note that the sulfonamides are not effective for the prophylaxis of infective endocarditis). • Primary prevention is not possible in patients who develop subclinical pharyngitis and therefore do not seek medical treatment (30%) and in patients who develop ARF without symptoms of strepto coccal pharyngitis (30%). • Oral erythromycin ethyl succinate. PRIMARY PREVENTION • Primary prevention of rheumatic fever is possible with a 10-day course of penicillin therapy for streptococcal pharyngitis. 250 mg.

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