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Acute Rheumatic Acute Rheumatic Fever Fever Dr. Basem Enany, MD Dr. Basem Enany, MD Lecturer of Cardiology Lecturer of Cardiology

management of acute rheumatic fever

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Page 1: management of acute rheumatic fever

Acute Rheumatic FeverAcute Rheumatic Fever

Dr. Basem Enany, MDDr. Basem Enany, MDLecturer of CardiologyLecturer of Cardiology

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The initial illness is usually characterized by a sore throat (pharyngitis) that may be followed, within approximately 1 to 5 weeks, by the sudden (acute) onset of rheumatic fever.

"latent period." ARF is an inflammatory disease following group A

streptococcal infection (i.e., sequelae) multiple tissues and organs (joints, skin, subcutaneous tissues, heart, and brain).

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Diagrammatic structure of the group A beta hemolytic streptococcus

Capsule

Cell wall

Protein antigens

Group carbohydrate

Peptidoglycan

Cyto.membrane

Cytoplasm

…………………………………………………...

Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain

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Evidence of AUTOIMMUNITY Evidence of AUTOIMMUNITY INDUCED BY STREPT. ANTIGENSINDUCED BY STREPT. ANTIGENS

Gamma-globulins in sarcolemma Gamma-globulins in sarcolemma of myofibrilsof myofibrils

Circulating ab. to heart tissue.Circulating ab. to heart tissue. No strept. can be found in No strept. can be found in

lesions.lesions.

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Not all of the serotypes of group A streptococci can cause rheumatic fever. The rheumatogenic serotypes are thought to include 1, 3, 5, 6, 14, 18, 19, and 24.

PharyngitisPharyngitis- - produced by GABHS can lead to- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonepritis& post strept. Glomerulonepritis

Skin infection-Skin infection- produced by GABHS leads to produced by GABHS leads to post streptococcal glomerulo nephritispost streptococcal glomerulo nephritis only. only.

Group A Beta Hemolytic Streptococcus

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INCIDENCEINCIDENCE 20 to 50 per 100,000 /year during the period of 1940 to

1960 and declined to 1/100,000/year in 1970s. 100/100,000/year of ARF/RHD among the younger age

group of the socially disadvantaged population.

THE ATTACK RATETHE ATTACK RATE(INCIDENCE OF ARF IN PTS WITH STREPT. PHARYNGITIS)(INCIDENCE OF ARF IN PTS WITH STREPT. PHARYNGITIS) 3% OF UNTREATED PATIENTS3% OF UNTREATED PATIENTS 5-50% IN PTS WITH PREVIOUS ATTACKS5-50% IN PTS WITH PREVIOUS ATTACKSEPIDEMIOLOGYEPIDEMIOLOGY SOCIO-ECONOMIC STATUSSOCIO-ECONOMIC STATUS OUT BREAKS OF STREPT PHARYNGITISOUT BREAKS OF STREPT PHARYNGITIS

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CARDIOVASCULAR LESIONSCARDIOVASCULAR LESIONS MYOCARDIUM (ASCHOFF BODY)MYOCARDIUM (ASCHOFF BODY) ENDOCARDIUMENDOCARDIUM PERICARDIUMPERICARDIUM

EXTRACARDIAC LESIONSEXTRACARDIAC LESIONS JOINTSJOINTS SKINSKIN LUNGS AND PLEURALUNGS AND PLEURA CNSCNS

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•On pathological examination, the valves are thickened and display rows of small vegetations along their apposing surfaces•Inflammation of the valves consists of oedema and mononuclear cell infiltration of the valvular tissue and the chordae tendineae in the acute phase; fibrosis and calcification occur with maintenance of the inflammatory process.

•Myocarditis is characterised by infiltration of mononuclear cells, vasculitis and degenerative changes of the interstitial connective tissue. •The pathognomonic lesion is the Aschoff body in the proliferative stage, present in 30 to 40 per cent of biopsies of patients with acute RF

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Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae

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Aortic valve showing active valvulitis. The valve is slightly thickened and displays small vegetations – "verrucae"

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•Stenotic mitral valve seen from left atrium.•Both commissures are fused; the cusps are severely thickened. •The left atrium is huge.

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Myocardial Aschoff body – the cells are large, elongated, with large nuclei; some are multinucleate

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Acute Rheumatic vegetationsAcute Rheumatic vegetations::

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Chronic RHDChronic RHD:: Valve leaflet Valve leaflet

thickening.thickening. Shortening, Shortening,

thickening and thickening and fusion of fusion of tendinous cords.tendinous cords.

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Fibrinous PericarditisFibrinous Pericarditis::

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Jones Criteria (Revised) for Guidance in theDiagnosis of Rheumatic Fever*

Major Manifestation MinorManifestations

Supporting Evidence of Streptococal Infection

Clinical LaboratoryCarditisPolyarthritis

ChoreaErythema Marginatum

Subcutaneous Nodules

Previousrheumaticfever orrheumaticheart diseaseArthralgiaFever

Acute phasereactants:Erythrocytesedimentationrate, C-reactiveprotein,leukocytosis Prolonged P-R interval

Increased Titer of Anti-Streptococcal Antibodies ASO (anti-streptolysin O),othersPositive Throat Culture for Group A StreptococcusRecent Scarlet Fever

*The presence of two major criteria, or of one major and two minor criteria,indicates a high probability of acute rheumatic fever, if supported by evidence ofGroup A streptococcal nfection.

Recommendations of the American Heart Association

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Pitfalls in diagnosisPitfalls in diagnosis JohnJohn’’s criteria is only a s criteria is only a guidelineguidelineProblems with over diagnosisProblems with over diagnosis A minor illness is misdiagnosed as ARF A minor illness is misdiagnosed as ARF unnecessarily unnecessarily

therapytherapy cardiac neuroticcardiac neuroticProblems with under diagnosisProblems with under diagnosis another disease another disease treatment for a non existent diseasetreatment for a non existent disease No long term prophylaxis No long term prophylaxis

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ARTHRITISARTHRITISmost commonmost common

IN 70% OF CASES ACUTE MIGRATORY ASYMMETRIC

POLYARTHRITIS USUALLY LARGE JOINTS Involved joint is swollen and exquisitely

painful and tender. RESOLVES WITHIN 1-3 WEEKS RESPONDS QUICKLY TO SALICYLATES,

this may be taken as a therapeutic test LEAVES NO PERMENANT DAMAGE

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PANCARDITISPANCARDITIS IN 50% OF CASESIN 50% OF CASES MOST SERIOUS CAUSE OF MORBIDITY AND MORTALITYMOST SERIOUS CAUSE OF MORBIDITY AND MORTALITY MAY BE THE ONLY MANIFESTATION OF ARFMAY BE THE ONLY MANIFESTATION OF ARF LEAVES PERMENANT DAMAGELEAVES PERMENANT DAMAGERheumatic carditis is pancarditis and endocardium is almost always Rheumatic carditis is pancarditis and endocardium is almost always

involved. Hence without murmur carditis cannot be diagnosed.involved. Hence without murmur carditis cannot be diagnosed.MYOCARDITIS:MYOCARDITIS:TACHYCARDIA,ARRHYTHMIAS,A-V BLOCKS, CARDIOMEGALY, CHFTACHYCARDIA,ARRHYTHMIAS,A-V BLOCKS, CARDIOMEGALY, CHFENDOCARDITIS:ENDOCARDITIS:MR,AR,TR,PR (STENOTIC LESIONS ONLY AFTER MONTHS OR YEARS)MR,AR,TR,PR (STENOTIC LESIONS ONLY AFTER MONTHS OR YEARS)With severe cardiac failure and pericardial effusion murmur may not With severe cardiac failure and pericardial effusion murmur may not

be audible but in such cases the patient is usually very ill.be audible but in such cases the patient is usually very ill.PERICARDITISPERICARDITIS:DRY OR WITH EFFUSION.NEVER ALONE.:DRY OR WITH EFFUSION.NEVER ALONE.

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Chest radiograph of an 8 year old patient with acute carditis before treatment

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Same patient after 4 weeks

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Two-dimensional color flow Doppler image of the left ventricular inflow of a patient with mitral regurgitation in the four-chamber view (top panel) and two-dimensional parasternal long-axis view (lower panel), showing lack of apposition of the leaflets of the mitral valve during systole (arrow)

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Two-dimensional parasternal long-axis view of a patient with mitral stenosis, showing thickened valve cusps (arrow), with poor leaflet separation in diastole. Left atrium is enlarged, with a thrombus in the posterior aspect of it. Aortic valve is also stenotic

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UNCOMMON (<10%), but most specificUNCOMMON (<10%), but most specific SMALL (0.5-2 cm.)SMALL (0.5-2 cm.) PAINLESS FIRM DISCRETE AND FREELY MOBILEPAINLESS FIRM DISCRETE AND FREELY MOBILE ON EXTENSOR TENDONS OF JOINTSON EXTENSOR TENDONS OF JOINTS OCCASIONALLY ON SCALP AND SPINEOCCASIONALLY ON SCALP AND SPINE The subcutaneous nodules tend to appear after The subcutaneous nodules tend to appear after

the first weeks of the disease course and the first weeks of the disease course and usually disappear within a week or two. usually disappear within a week or two.

Subcutaneous nodules

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Subcutaneous nodule on the extensor surface of elbow of a patient with acute RF

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Sydenham's chorea most frequently occurs in children or adolescents between the ages of 5 to 15.

Affects females approximately twice as frequently as males, particularly in the years around puberty. As a result, some researchers suggest that sex hormones (e.g., the female hormone estrogen) may play some role in the development of the syndrome.

CHOREA

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LONG LATENT PERIOD:LONG LATENT PERIOD: 1 to 6 months 1 to 6 months In most patientsIn most patientsacutelyacutely sudden, aimless, irregular, involuntary, jerky sudden, aimless, irregular, involuntary, jerky

movementsmovements A significant deterioration in handwriting (in school-aged A significant deterioration in handwriting (in school-aged

children) children) Slight or significant difficulties dressing, feeding, and walking Slight or significant difficulties dressing, feeding, and walking Slurred, slowed speech (dysarthria) Slurred, slowed speech (dysarthria)

disappear disappear with sleep and maywith sleep and may increase increase with stress, with stress, fatigue, excitement, or other factors.fatigue, excitement, or other factors.

BilateralBilateral (20% hemichorea) (20% hemichorea) emotional or behavioral abnormalitiesemotional or behavioral abnormalities spontaneously resolve within approximately spontaneously resolve within approximately 3 to 6 months3 to 6 months However, in some instances, there may be residual signs of However, in some instances, there may be residual signs of

chorea and behavioral abnormalities, which may wax and wane chorea and behavioral abnormalities, which may wax and wane over a year or moreover a year or more

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RARE (5-10%) MACULAR NONPRURITIC RASH WITH A

SERPIGINOUS ERYTHEMATOUS BORDER SURROUNDING NORMAL LOOKING SKIN

BEGINS AS RED OR PINK MACULES THAT FADE CENTRALLY

ON TRUNK & PROXIMAL EXTREMITIES NEVER FACE AND HANDS ABOUT 1INCH IN DIAMETER This skin rash tends to appear early in the disease

course, may persist or recur when other symptoms have subsided, and usually only affects patients with carditis.

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Erythema marginatum on the trunk, showing erythematous lesions with pale centers and rounded or serpiginous margins

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LABORATORY STUDIESLABORATORY STUDIES

ISOLATION OF STREPT. (THROAT ISOLATION OF STREPT. (THROAT CULTURES)CULTURES)

Throat culture render positive Throat culture render positive results in approximately 25 % of results in approximately 25 % of children of ARF probably related children of ARF probably related to early antibiotic administration. to early antibiotic administration.

-VE(75% OF PTS.)-VE(75% OF PTS.) FALSE +VE: FALSE +VE: Positive throat culture Positive throat culture

need not indicate infection because need not indicate infection because positive throat culture may occur in carrier positive throat culture may occur in carrier state as in many school going children.state as in many school going children.

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STREPTOCOCCAL AB. TESTSSTREPTOCOCCAL AB. TESTS

ANTIGENANTIGENEXTRACELLULAR PRODUCTEXTRACELLULAR PRODUCT

• SREPTOLYSIN-OSREPTOLYSIN-O• SREPTOKINASESREPTOKINASE• HYALURONIDASEHYALURONIDASE• DEOXYRIBONUCLEASE -NDEOXYRIBONUCLEASE -N• NICOTINAMIDE ADENINE NICOTINAMIDE ADENINE

DINUCLEOTIDASEDINUCLEOTIDASE• ALL OF THE ABOVEALL OF THE ABOVE

CELLULAR COMPONENTCELLULAR COMPONENT• TYPE-SPECIFIC M PROTEINTYPE-SPECIFIC M PROTEIN• GROUP-SPECIFIC POLYSACCHARIDEGROUP-SPECIFIC POLYSACCHARIDE

TESTTEST ANTI-STREPTOLYSIN-0ANTI-STREPTOLYSIN-0 ANTI-STREPTOKINASEANTI-STREPTOKINASE ANTI-HYALURONIDASEANTI-HYALURONIDASE ANTI-DNAse BANTI-DNAse B ANTI-NADaseANTI-NADase STREPTOZYMESTREPTOZYME

TYPE-SPECIFIC AB.TYPE-SPECIFIC AB. ANTI-A CARBOHYDRATEANTI-A CARBOHYDRATE

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positive positive ASOTASOT occur only in 80 % of occur only in 80 % of streptococcal throat infection. However streptococcal throat infection. However sensitivity may be increased to 95 % if sensitivity may be increased to 95 % if AHTAHT and and anti DN ase Banti DN ase B are also tested. are also tested.

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OTHER INVESTIGATIONSOTHER INVESTIGATIONSCXRCXR CARDIC SIZE(CHF,EFFUSION)CARDIC SIZE(CHF,EFFUSION) RHC. PNUEMONITISRHC. PNUEMONITISECGECG SINUS TACHCARDIASINUS TACHCARDIA PROLONGED P-RPROLONGED P-R ARRHYTHMIASARRHYTHMIAS ST-T CHANGESST-T CHANGESECHOCARDIOGRAPHYECHOCARDIOGRAPHY MYOCARDIAL(DILATATION,FAILURE)MYOCARDIAL(DILATATION,FAILURE) PERICARDIAL(EFFUSION)PERICARDIAL(EFFUSION) ENDOCARDIAL(VALVULAR AFFECTION)ENDOCARDIAL(VALVULAR AFFECTION)

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DIFFERENTIAL DIAGNOSISDIFFERENTIAL DIAGNOSIS

POLYARTHRITISPOLYARTHRITIS JUVENILE RHEUMATOID: usually involves small joints of the fingers and JUVENILE RHEUMATOID: usually involves small joints of the fingers and

here the swelling is disproportionate to the symptom and usually the here the swelling is disproportionate to the symptom and usually the manifestation takes a longer time to subsides and residual deformity is manifestation takes a longer time to subsides and residual deformity is common.common.

‘‘Growing painsGrowing pains’’ of children is mistaken for arthritis. But the symptom is of children is mistaken for arthritis. But the symptom is not over the joints, pain is severe at night and the child is well during not over the joints, pain is severe at night and the child is well during the day time. the day time.

SLESLE MIXED COLLAGEN DSE.MIXED COLLAGEN DSE. POST-INFECTIOUS REACTIVEPOST-INFECTIOUS REACTIVE INFECTIVE INFECTIVE SERUM SICKNESSSERUM SICKNESS

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D.D. of CARDITISD.D. of CARDITIS Innocent murmurs:Innocent murmurs: The common mistake is The common mistake is

misinterpreting the innocent basal ejection systolic murmur misinterpreting the innocent basal ejection systolic murmur or left parasternal systolic murmur (Stillor left parasternal systolic murmur (Still’’s) as evidence of s) as evidence of carditis since they are misinterpreted for mitral carditis since they are misinterpreted for mitral regurgitation. Stillregurgitation. Still’’s murmur is vibratory in quality, usually s murmur is vibratory in quality, usually late systolic unlike the systolic murmur of carditis which is late systolic unlike the systolic murmur of carditis which is usually pansystolic or occupies most of systole. The quality usually pansystolic or occupies most of systole. The quality is also different from Stillis also different from Still’’s murmur. Isolated ejection s murmur. Isolated ejection systolic murmurs shall never be taken as evidence of systolic murmurs shall never be taken as evidence of carditis. carditis.

Tachycardia associated with fever and anxietyTachycardia associated with fever and anxiety may may be misinterpreted as evidence of myocarditis. This can be be misinterpreted as evidence of myocarditis. This can be avoided if one pays attention to sleeping pulse rate. avoided if one pays attention to sleeping pulse rate.

INFECTIVE ENDOCARDITISINFECTIVE ENDOCARDITIS COLLAGEN DSE.(SLE,KAWASAKI)COLLAGEN DSE.(SLE,KAWASAKI) VIRAL MYOCARDITIS/pericarditisVIRAL MYOCARDITIS/pericarditis

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TreatmentTreatment Step IStep I - primary prevention - primary prevention

(eradication of streptococci)(eradication of streptococci) Step IIStep II - anti inflammatory treatment - anti inflammatory treatment

(aspirin,steroids)(aspirin,steroids) Step IIIStep III- supportive management & - supportive management &

management of complications management of complications Step IVStep IV- secondary prevention - secondary prevention

(prevention of recurrent (prevention of recurrent attacks)attacks)

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STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)

Agent Dose Mode DurationBenzathine penicillin G 600 000 U for patients< IM Once

27 kg (60 lb) 1 200 000 U for patients >27 kg

or Penicillin V Children: 250 mg 2-3 times daily Oral 10 d (phenoxymethyl penicillin) Adolescents and adults:

500 mg 2-3 times daily

For individuals allergic to penicillinErythromycin: 20-40 mg/kg/d 2-4 times daily Oral 10 d

(maximum 1 g/d)

Recommendations of American Heart Association

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Arthritis only Aspirin 75-100 mg/kg/day,give as 4 divided doses for 6 weeks (Attain a blood level 20-30 mg/dl)

Carditis Prednisolone 2-2.5 mg/kg/day, give as two divided doses for 2 weeks Taper over 2 weeks & while tapering add Aspirin 75 mg/kg/day for 2 weeks. Continue aspirin alone 100 mg/kg/day for another 4 weeks

Step II: Anti inflammatory treatmentClinical condition Drugs

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Bed rest Bed rest Treatment of congestive cardiac Treatment of congestive cardiac

failure: -failure: -digitalis,diuretics, ACEIdigitalis,diuretics, ACEI Treatment of chorea:Treatment of chorea:

--diazepam or haloperidoldiazepam or haloperidol Rest to joints & supportive splintingRest to joints & supportive splinting

3.Step III: Supportive management & management of complications

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STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)

Agent Dose Mode

Benzathine penicillin G 1 200 000 U every 4 weeks* Intramuscular

orPenicillin V 250 mg twice daily Oral

orSulfadiazine 0.5 g once daily for patients 27 kg (60 lb Oral

1.0 g once daily for patients >27 kg (60 lb)

For individuals allergic to penicillin and sulfadiazine

Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified and recommended

Recommendations of American Heart Association

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Duration of Secondary Rheumatic Fever Prophylaxis

Category DurationRheumatic fever with carditis and At least 10 y since last residual heart disease episode and at least until (persistent valvar disease*) age 40 y, sometimes lifelong

prophylaxis

Rheumatic fever with carditis 10 y or well into adulthood, but no residual heart disease whichever is longer (no valvar disease*)

Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer

*Clinical or echocardiographic evidence.

Recommendations of American Heart Association

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SYDENHAMSYDENHAM’’S CHOREAS CHOREA PHYSICAL & MENTAL RESTPHYSICAL & MENTAL REST As Sydenham's chorea may spontaneously As Sydenham's chorea may spontaneously

resolve or not cause significant functional resolve or not cause significant functional impairment, many experts indicate that impairment, many experts indicate that treatmenttreatment should be should be avoidedavoided unless unless associated chorea is associated chorea is functionally disabling functionally disabling or associated with potentially violent or associated with potentially violent flailing motions of the limbs that may result flailing motions of the limbs that may result in in self-injuryself-injury. .

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First-line therapy with anticonvulsant First-line therapy with anticonvulsant medication:medication: valproate sodium valproate sodium (Depakene(Depakene®®) may be beneficial) may be beneficial

Carbamazepine Carbamazepine has also been suggested has also been suggested as a first-line treatment for Sydenhamas a first-line treatment for Sydenham’’s s chorea.chorea.

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Dopamine antagonistsDopamine antagonists are usually reserved for are usually reserved for those patients who fail to respond to valproate or those patients who fail to respond to valproate or who present with severe forms (i.e., chorea who present with severe forms (i.e., chorea paralytica). paralytica).

Haloperidol (initial dose of 0.5 to 1mg/kg/day, Haloperidol (initial dose of 0.5 to 1mg/kg/day, maximum, 5mg/day) maximum, 5mg/day)

If fails,If fails, the next steps may include the next steps may include immunomodulatory treatment, steroids, IV IgG, or immunomodulatory treatment, steroids, IV IgG, or plasmapheresis. plasmapheresis.

Treatment is usually maintained for 8-12 Treatment is usually maintained for 8-12 weeks.weeks.

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ARF IS THE MOST COMMON CAUSE OF ARF IS THE MOST COMMON CAUSE OF ACQUIRED HEART DISEASE IN CHILDREN ACQUIRED HEART DISEASE IN CHILDREN AND YOUNG ADULTS.AND YOUNG ADULTS.

DIAGNOSIS OF ARF SHOULD DEPEND ON DIAGNOSIS OF ARF SHOULD DEPEND ON CLINICAL,LABORATORY & IMAGING CLINICAL,LABORATORY & IMAGING INVESTIGATIONS.INVESTIGATIONS.

TREATMENT OF CARDITIS WITH SALICYLATES TREATMENT OF CARDITIS WITH SALICYLATES , STEROIDS., STEROIDS.

LONG-TERM PROPHYLAXIS WITH LONG LONG-TERM PROPHYLAXIS WITH LONG ACTING PCN. IS HIGHLY RECOMMENDED.ACTING PCN. IS HIGHLY RECOMMENDED.