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Acute Rheumatic Fever

Muhammad Ali Pediatric Cardiology Division University of Sumatera Utara

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

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

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

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

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

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

• 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. weakness. • The adventitious movements. • Recently. and hypotonia continue for an average of 7 months (up to 17 months) before slowly waning in severity. . The levels of the antineuronal antibody titer are positively related to the severity of choreic movements.

. • Fever (usually with a temperature of at least 102°F [38. elevated acute-phase reactants (elevated C-reactive protein levels and elevated erythrocyte sedimentation rate) are objective evidence of an inflammatory process.8°C]) is present early in the course of untreated rheumatic fever. • A prolonged PR interval on the ECG is neither specific for ARF nor an indication of active carditis. • In laboratory findings.MINOR MANIFESTATIONS • Arthralgia refers to joint pain without the objective changes of arthritis. 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. • 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. a negative test result should be confirmed by a conventional throat culture. The onset of the clinical manifestations of ARF coincides with the peak of the streptococcal antibody response. . Antigen detection tests are very specific but not very sensitive.

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

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

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

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

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

. and does not cause permanent damage. even without treatment.Clinical Course • Only carditis can cause permanent cardiac damage. but those of severe carditis may last for 2 to 6 months. • Chorea gradually subsides in 6 to 7 months or longer and usually does not cause permanent neurologic sequelae. Signs of mild carditis disappear rapidly in weeks. • Arthritis subsides within a few days to several weeks.

0. . ASO titer. may be substituted for penicillin.Management • When ARF is suggested by history and physical examination. throat culture. erythromycin. is given to eradicate streptococci. 40 mg/kg per day in two to four doses for 10 days. acute-phase reactants (ESR and CRP).2 million IU/IM. In patients allergic to penicillin. two-dimensional echo and Doppler studies are usually performed at that time.6 to 1. • Benzathine penicillin G. one should obtain the following laboratory studies: complete blood count. This serves as the first dose of penicillin prophylaxis as well. chest x-ray films. and ECG. • Cardiology consultation is indicated to clarify whether there is cardiac involvement.

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

• 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.

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

• For arthritis. • • For mild to moderate carditis. An adequate blood level of salicylates is 20 to 25 mg/100 mL. depending on the clinical response. After improvement. the therapy is with drawn gradually over 4 to 6 weeks while monitoring acute-phase reactants. This dose is continued for 4 to 8 weeks. Rapid resolution of joint symptoms with aspirin within 24 to 36 hours is supportive evidence of the arthritis of ARF. . • Prednisone (2 mg/kg per day in four divided doses for 2 to 6 weeks) is indicated only in cases of severe carditis. aspirin alone is recommended in a dose of 90 to 100 mg/kg per day in four to six divided doses. aspirin therapy is continued for 2 weeks and gradually withdrawn over the following 2 to 3 weeks.• Therapy with anti-inflammatory agents should be started as soon as ARF has been diagnosed.

if indicated. beginning with half the usual recommended dose.Treatment of CHF includes some or all of the following: • Complete bed rest with orthopneic position and moist. 1 mg/kg every 6 to 12 hours. . • Restriction of sodium and fluid intake. because certain patients with rheumatic carditis are supersensitive to digitalis). cool oxygen. • Furosemide. • Digoxin (used with caution. • Prednisone for severe carditis of recent onset. 0. • Morphine sulfate. at 4-hour intervals for severe CHF with respiratory distress.2 mg/kg.

Without the prophylaxis. any of the following drugs may be used: phenobarbital (15 to 30 mg every 6 to 8 hours). – – – . Give benzathine penicillin G. haloperidol (starting at 0.2 million units. just as in patients with other rheumatic manifestations. 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). valproic acid. chlorpromazine (Thorazine). initially for eradication of streptococcus and also every 28 days for prevention of recurrence. 1. about 25% of patients with isolated chorea (without carditis) develop rheumatic valvular heart disease in 20year follow-up. but the preliminary results are promising in reducing the duration of chorea.5 mg and increasing every 8 hours to 2 g). Anti-inflammatory agents are not needed in patients with isolated chorea. diazepam (Valium). or steroids.Management of Sydenham's chorea: – – Reduce physical and emotional stress and use protective measures as indicated. For severe cases.

Valvular disease resolves more frequently when prophylaxis is followed. 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. . the greater the incidence of residual heart disease. • 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. • 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.

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

1. • Oral erythromycin ethyl succinate. twice daily. 250 mg. • 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%).2 million units given intramuscularly every 28 days (not once a month). . • Oral sulfadiazine.METHODS The method of choice for secondary prevention is benzathine penicillin G. are the following: • Oral penicillin V. Alternative methods. PRIMARY PREVENTION • Primary prevention of rheumatic fever is possible with a 10-day course of penicillin therapy for streptococcal pharyngitis. 250 mg. twice daily. 1 g once daily (note that the sulfonamides are not effective for the prophylaxis of infective endocarditis). although not as effective.

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