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Acute Rheumatic Fever (ARF)
Iraj Salehi-Abari, MD., Internist Rheumatologist
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ARF Introduction: I. Salehi A. A delayed, nonsuppurative sequela of
A pharyngeal Strep. infection( GAS ), with a Latent period of 2 to 3 weeks( 1 -5 weeks ) With fever & cardinal manifestations of: Arthritis, Carditis, Chorea Subcutaneous nodules Erythema marginatum I. Salehi A.
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ARF I. Salehi A.
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ARF Epidemiology: I. Salehi A. Worldwide
Although a dramatic decline in both the severity & mortality of ARF has occurred since the turn of the century, recent reports have documented its resurgence in US New cases/ year: 470,000 I. Salehi A.
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ARF Epidemiology: I. Salehi A. Death/ year: 233,000 due to ARF or RHD
Most occur in developing countries Incidence: Mean: 19/ 100,000 In US & developed countries: 2 to 14/ 100,000 Most common in children: Four to 9 years of age I. Salehi A.
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ARF Pathogenesis: I. Salehi A. Incompletely understood
Streptococcal Pharyngitis with Rheumatogenic strains: M serotype: 3, 5, 6, 14, 18, 19, 24 Genetic( Host ) susceptibility Strep. Toxins are not important Molecular mimicry I. Salehi A.
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Role of the Streptococcus:
ARF Role of the Streptococcus: No direct involvement of GAS in tissues Outbreaks of ARF follow epidemics of Strep. Pharyngitis Treatment of Strep. Pharyngitis Reduces the incidence of ARF AB prophylaxis of Strep. Pharyngitis Prevents the recurrence of ARF I. Salehi A.
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Role of the Streptococcus:
ARF Role of the Streptococcus: Most patients with ARF have elevated titers to at least one of three anti-Strep. Antibodies: ASO, AH, ASK ARF might be due to non-GAS eg C & G that have GAS antigens or enzymes I. Salehi A.
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Importance of pharyngitis
ARF Importance of pharyngitis Only Strep. Pharyngitis associated with ARF Impetigo can cause GN but almost never ARF Pharyngeal strains: A, B, C Impetigo strains: D, E If impetigo & ARF were concomitant Subclinical pharyngitis Strains colonizing the skin were different Sometimes impetigo is due to GAS Pharyngitis may be due to non-GAS with GAS Ag I. Salehi A.
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Importance of pharyngitis:
ARF Importance of pharyngitis: Factor affecting localization to pharynx: CD44, a hyaluronic acid binding protein act as A pharyngeal receptor for GAS GAS can not colonize oropharynx in transgenic mice that do not express CD44 Pharynx has large repository of lymphoid tissue I. Salehi A.
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ARF Molecular mimicry: I. Salehi A.
Antibodies directed against GAS antigens crossreact with host antigens Carditis: Strep. M protein / myosin Strep. NABG / myosin Chorea: Strep. NABG / lysoganglioside I. Salehi A.
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Genetic susceptibility:
ARF Genetic susceptibility: B cells marked with D8/17 D8/17 > 20% of B cell in 100% of ARF D8/17 + B cells of 4-6% in 95% of NL population HLA-DR4 & DR2: Caucasian & Black HLA-DR1 & DRW6: South African black HLA-DR7 & DW53 I. Salehi A.
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Clinical Manifestations:
ARF Clinical Manifestations: An acute febrile illness in children within 1 to 5 weeks after pharyngitis Manifest in one of several ways: Migratory polyarthritis, large joints Carditis and valvulitis CNS involvement, Sydenham chorea Rash & nodules Some combination of the above I. Salehi A.
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ARF Arthritis: I. Salehi A. Migratory polyarthritis of large joints
Knee, Ankle, Elbow, Wrist Self-limited, non-erosive, non-deformans Duration of < 1 week Usually is the earliest manifestation Joint pain > objective signs of arthritis I. Salehi A.
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ARF Arthritis: I. Salehi A.
Dramatically responsive to aspirin & NSAIDs Monoarthritis: until 17% Chronicity & jaccoud deformity: 5% Synovial fluid: Sterile, inflammatory DD with poststrep. Reactive arthritis( PSRA ) I. Salehi A.
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Migratory polyarthritis:
ARF Migratory polyarthritis: ARF Gonoccocal arthritis SLE Viral arthritis Rubella Hepatitis B Sarcoidosis I. Salehi A.
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ARF Jaccoud deformity: ARF SLE Parkinson Sarcoidosis I. Salehi A.
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ARF Carditis: With more sensitive Echo: nearly all patients with ARF have acute carditis But often subtle( Subclinical ) Pancarditis Pericarditis Endocarditis Myocarditis I. Salehi A.
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Pericarditis: ARF Chest discomfort Pleuritic chest pain
Pericardial friction rub Pericardial effusion in Echo ST-T changes in ECG I. Salehi A.
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Endocarditis: ARF New or changing murmurs Pansystolic murmur( MR )
Basal diastolic decrescendo murmur Systolic ejection murmur Mid-diastolic murmur( Carey-Coombs) I. Salehi A.
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Myocarditis: ARF CHF Arrhythmia Heart block Sudden cardiac death
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ARF Carditis I. Salehi A. Heart Failure: MR: most common
The most life-threatening Severe valvular damage &/or myocarditis MR: most common ECG: All degree of heart block, AV dissociation CXR: cardiomegaly Echocardiography Antimyosin scintigraphy I. Salehi A.
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ARF I. Salehi A.
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ARF Chorea I. Salehi A. The different names:
Sydenham chorea Chorea minor St. Vitus dance A neurologic disorder consisting of: Abrupt, purposeless, nonrhythmic involuntary movement Muscular weakness Emotional disturbances I. Salehi A.
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ARF Chorea I. Salehi A. Cease during sleep
Relapsing grip or ‘‘milking sign’’ Inappropriate behavior Crying Restlessness Transient psychosis: rare I. Salehi A.
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ARF Chorea I. Salehi A. A longer latent period after Strep. infection
May be alone manifestation May be accompanied by heart murmurs Neurologic examination: No sensory losses No pyramidal tract involvement Diffuse hypotonia may be present I. Salehi A.
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Subcutaneous nodules:
ARF Subcutaneous nodules: Firm, painless Overlying skin is not inflamed, so movable Over bony surface or near tendons Different size & number Occur in < 5% of patients After the first week Usually occur with carditis I. Salehi A.
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ARF I. Salehi A.
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ARF Erythema marginatum: I. Salehi A.
An evanescent, non-pruritic, pink rash Trunk, proximal limbs Erythema annulare Reveal with hot bath or shower Early manifested Occur with carditis I. Salehi A.
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ARF I. Salehi A.
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ARF Diagnosis: I. Salehi A. There is not Diagnostic Lab. Test
Diagnosis is clinical and Lab. Tests are supporting Jones criteria( the 1992 revision ): Major criteria Minor criteria Supporting evidence of a preceding GAS infection I. Salehi A.
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ARF Major Criteria: I. Salehi A. Carditis Polyarthritis Chorea
Erythema marginatum Subcutaneous nodules I. Salehi A.
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ARF Minor Criteria: I. Salehi A. Clinical findings:
Arthralgia Fever Laboratory findings: Elevated ESR, CRP Prolonged PR interval I. Salehi A.
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ARF Supporting Criteria: I. Salehi A.
Positive throat culture for GAS or Positive rapid Strep. Antigen test Elevated or rising Strep. Antibody titer, most often ASO I. Salehi A.
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Diagnosis by Jones Criteria:
ARF Diagnosis by Jones Criteria: Two major criteria + One Supporting Criteria or One major criteria + Two minor criteria + One Supporting criteria I. Salehi A.
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ARF Exceptions: I. Salehi A. Monoarthritis + Fever
Chorea as the only manifestation Carditis months after the acute infection Recurrent Rheumatic fever in patients with Hx of Previous ARF or RHD In above states one major or two minor criteria + supporting evidences are Diagnostic I. Salehi A.
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ARF 2002 AHA update: I. Salehi A.
The major conclusion was that there were insufficient data to support a revision of the 1992 criteria Strict adherence to the jones criteria in areas of high prevalence may result in underdiagnosis Clinical findings assume increased importance in regions where microbiologic & immunologic tests are not available I. Salehi A.
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ARF 2002 AHA update: I. Salehi A.
Concluded that Doppler Echo. Findings alone should not be either a major or minor jones Criterion for the diagnosis of ARF Opposing opinions: The valve regurgitation being a major criterion in patients with isolated chorea or polyarthritis I. Salehi A.
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ARF Laborory testing: I. Salehi A. Throat cultures:
are negative in 75% of patients by the time ARF appears Streptococcal antibodies: They reach a peak titer at about the time of onset of ARF They indicate true infection rather than transient carriage By several tests any recent GAS infection can be detected I. Salehi A.
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ARF Laborory testing: I. Salehi A. Antistreptolysin O( ASO ):
In healthy children of school age have titers of 200 to 300 Todd u/ml Peaks at 4-5 weeks after pharyngitis or 2ed-3rd weeks of ARF It is useful to take two ASO tests, one at first and another one 2 weeks later ASO titers fall off rapidly in the next several months & reach a slower decline after 6 months I. Salehi A.
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ARF Laborory testing: I. Salehi A. Antistreptolysin O( ASO ):
Titers can not be used as a measure of rheumatic activity Pharyngeal Strep. Carriers have very low titers, just above detectable Only 80% of patients have rised titer of ASO I. Salehi A.
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ARF Laborory testing: I. Salehi A. Other Anti-Strep. Antibodies:
Anti-DNAse B Antistreptokinase Antihyaluronidase Other Lab. Data: ESR, CRP AOCD I. Salehi A.
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Rheumatic Heart Disease( RHD )
ARF Rheumatic Heart Disease( RHD ) The most severe sequela of ARF It occurs yrs after original attack of ARF The major cause of acquired valvular disease It occurs in 50% of patients with carditis M.v > A.v, MS is the classic finding I. Salehi A.
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ARF Treatment: I. Salehi A. Three major goals in the treatment:
Symptomatic relief of ARF Eradication of GAS infection Prophylaxis against future infection to prevent recurrent cardiac disease I. Salehi A.
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ARF Symptomatic relief: I. Salehi A.
Arthritis & Fever & mild Carditis: Aspirin, NSAIDs, Corticosteroids( LDS ) Dramatic improvement Until Ph. Ex. & ESR , CRP are normal Rash & Nodules Not need treatment I. Salehi A.
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Treatment of Carditis:
ARF Treatment of Carditis: Severe carditis: Cardiomegaly CHF CHB Treatment of severe carditis: Corticosteroid( HDS ) + Treatment of heart failure Surgery I. Salehi A.
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Eradication of GAS infection:
ARF Eradication of GAS infection: Benzathine Penicillin G: One single IM dose of 600,000 units for < 27kg 1,200,000 units for > 27 kg Penicillin V : Ten days oral dose of 250 mg TDS daily for children 500 mg TDS daily for adults I. Salehi A.
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Eradication of GAS infection:
ARF Eradication of GAS infection: In patients who allergic to penicillin: Erythromycin; 40 mg/kg/day for 10 days Oral Cephalosporin Throats culture of all family contacts: If positive for GAS Penicillin therapy is initiated I. Salehi A.
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Antibiotic Prophylaxis:
ARF Antibiotic Prophylaxis: Primary : Prevention of ARF Secondary: Prevention of recurrency of ARF Tertiary: SBE prophylaxis in patients with RHD I. Salehi A.
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Antibiotic Prophylaxis:
ARF Antibiotic Prophylaxis: Penicillin IM: Every 3 – 4 weeks Oral: 250 mg twice daily Sulfadiazine 500 mg/day for < 27 kg 1000 mg/day for > 27 kg Erythromycin 250 mg twice daily I. Salehi A.
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Secondary prevention:
ARF Secondary prevention: WHO guidelines : No proven Carditis: > 5 years or until age 18 Mild Carditis: mild MR > 10 years or until age of 25 Severe Carditis: Severe valve disease or valve surgery Life long prophylaxis I. Salehi A.
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