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ECHO

http://info.med.yale.edu/intmed/cardio/echo_a tlas/entities/mitral_regurgitation.html

Acute Rheumatic Fever

Danielle Cherian Morning Report August 2005

Epidemiology

1889 ARF was at its most prevalent and virulent and diagnostic criteria developed 1944 Jones Criteria published Declining pattern of incidence in US until mid-1980s, resurgence since 1984 1992 Jones Criteria reaffirmed and revision made with update focusing on evidence of a preceding strep infection Prevalence rate in developing countries up to 24/1000 Prevalence rate worldwide varies 0.5-11/1000 Rheumatic fever occurs most frequently among children and adolescents between 5 and 18 years, coinciding with the age distribution of the highest prevalence of streptococcal infections

Diagnostic Criteria
Major Criteria Carditis Polyarthritis Chorea Subcutaneous nodules Erythema marginatum
* 2 major or 1 major + 2 minor

Minor Criteria Fever Arthralgias Elevated CRP, ESR Prolonged PR interval

PLUS EVIDENCE OF ACUTE STREP INFECTION:THROAT CULTURE, RAPID ANTIGEN TEST, STREP Ab TITERS

Limitations to Criteria

Indolent carditis may first bring patient to medical attention, several months into rheumatic attack Chorea is the last major sign to appear Late in the course, no direct evidence of previous strep infection (ab titers have decreased, throat cx negative, acute phase reactants normalized) Recurrent rheumatic fever

CLINICAL MANIFESTATIONS

Group A streptococcus pharyngeal infection 2-4 weeks before onset of ARF symptoms Migratory arthritis involving large joints Carditis and valvulitis Chorea Erythema marginatum Subcutaneous nodules

Arthritis

Observed in 60-80% of patients with ARF Several joints in quick succession Knees, ankles, elbows, wrists most common Migrates from joint to joint Joint involvement is more common and more severe in adolescents/young adults Earliest symptom of ARF Exquisitely tender, few objective findings Radiographs normal NSAIDS may alter course

Carditis

RF produces a pancarditis, invariably associated with a valvulitis Can be easily diagnosed by auscultation, currently echo used to confirm findings New or changing murmurs MR is most common finding (murmur heard best at apex, moderate-to-high intensity, holosystolic, radiates to axilla, does not change with respiration or position) Aortic valve frequently associated with mitral (murmur of aortic insufficiency is soft, diastolic, high pitched and blowing, decreases in intensity toward end of diastole) Right heart valves rarely affected

Carditis

Most severe clinical manifestation of RF Severe valve damage and myocarditis can lead to severe heart failure and death Occurs in approximately 40-50% of patients Mitral stenosis is a manifestation of late scarring and calcification of damaged valves ECG abnl can include all forms of heart block CXR may show cardiomegaly

Carditis - Pathology

Valves thickened with rows of small vegetations Myocarditis is infiltration of mononuclear cells, vasculitis and degenerative changes of the interstitial connective tissue The pathognomonic lesion is the Aschoff body, present in 30 to 40% of biopsies, an agglomeration of monocytes/macrophages and B cells acting as APCs Inflammation of the valves consists of edema and mononuclear cell infiltration Inflammation of chordae tendineae in the acute phase Fibrosis and calcification occur with maintenance of the inflammatory process

Chorea

Sydenham chorea is a neurologic disorder consisting of abrupt, purposeless, nonrhythmic involuntary movements, muscular weakness, and emotional disturbances Movements commonly more marked on one side and cease during sleep Muscle weakness revealed by relapsing grip Emotional changes manifest as outbursts of inappropriate behavior, rarely transient psychosis Diffuse hypotonia may be present Longer latent period after strep infection

Subcutaneous Nodules

Firm and painless nodules Overlying skin is not inflamed and can be moved over the nodules Size varies from 2 mm to 2 cm Nodules located over bony surfaces Number of lesions vary from single to dozen, average 3-4; may be symmetric Elbows more frequently involved Smaller and more short-lived than those of RA Appear in first few weeks of illness Occurs only in patients with carditis

Erythema Marginatum

Evanescent, non-pruritic rash, pink or faintly red, affecting the trunk, proximal limbs and spares the face Erythematous lesions with pale centers and serpiginous margins Margin of lesions are continuous and make a ring, also known as erythema annulare Individual lesions appear and disappear in a matter of hours Occurs early in disease and persists when all other manifestations have disappeared Occurs only in patients with carditis

Pharyngitis

Streptococcal pharyngitis has been the only documented strep infection associated with ARF Impetigo can cause glomerulonephritis but almost never ARF Rheumatogenic strains issue unresolved some studies suggest that extrapharyngeal infections with GAS (pyodermas, soft-tissue infx) are not rheumatogenic Nephritogenicity is a property of only certain strains of GAS, mostly strains affecting the skin

Pathogenesis

Incompletely understood Streptococcal pharyngitis is required and ARF is a postinfectious sequela Autoimmune response to epitopes in the organism that are immunologically crossreactive with similar epitopes in human tissues (joints, heart, brain, skin) Genetic susceptibility may be present

Treatment

Symptomatic relief ASA 80-100 mg/kg/day for arthritis Until symptoms absent and ESR, CRP normal Corticosteroids for carditis Studies have shown conflicting results on effects PO prednisone 2mg/kg/day for 1-2 weeks then taper Eradication of GAS PCN 250mg TID (child) or 500mg TID x 10 days IM PCN should be given if compliance is an issue 600,000 units for children < 27kg 1.2 million units for children > 27kg and adults Prevent Recurrence Common within 2 years of original attack, anytime Antibiotic prophylaxis should be started immediately after resolution of acute illness PO PCN BID or IM PCN 1.2 mil units Q 3-4 weeks Duration unclear: 10 years from acute attack Lifetime prophylaxis for carditis