AM Report March 5, 2010 Amy Auerbach Acute Rheumatic Fever AM Report March 5, 2010 Amy Auerbach

Predisposing Factors Typically preceded by group A streptococcal tonsillopharyngitis (two to four weeks prior to ARF) ? Whether GAS skin infections could also predispose Most frequent in children between 4 and 9 years of age

Clinical Manifestations Migratory arthritis (involving large joints) Carditis and valvulitis Central nervous system involvement (Sydenham chorea) Rash

Arthritis Typically migratory arthritis- knees, ankles, elbows, wrists most commonly Often earliest manifestation May see monoarticular arthritis if patient has received salicylates or other NSAIDs Joint analysis demonstrates steril inflammatory fluid Minimal changes in serum levels of complement

Carditis Pancarditis affecting the pericardium, epicardium, myocardium, and endocardium Symptoms may be subtle or non-existent May see mitral regurgitation early on Mitral stenosis is manifestation of late scarring and calcification Echocardiography always indicated to assess for valvular disease

Chorea Sydenham chorea- abrupt, purposeless, nonrhythmic involuntary movements, muscular weakness, emotional disturbances Commonly more marked on one side and cease during sleep Emotional disturbances typically manifest as inappropriate behavior, crying, restlessness Can see diffuse hypotonia Often has longer latent period than other manifestations

Subcutaneous Nodules Firm and painless, typically a few milliliters to one to two centimeters Typically located over bony surface or near tendon Present for 1-2 weeks but rarely more than a month Least common manifestation (in the US)

Erythema marginatum Typically occurs early in the disease May persist or recur even when all other manifestations have disappeared Typically occurs only in patients with carditis

Jones Criteria High probability of ARF if the following criteria are met: Evidence of preceding group A streptococcal infection Two major manifestations OR One major and two minor manifestations

Major Manifestations Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules

Minor Manifestations Arthralgia Fever Elevated ESR or CRP Prolonged PR interval

Other Factors Three settings in which diagnosis can be made without Jones Criteria Chorea as only manifestation Indolent carditis months after acute infection Recurrent rheumatic fever in patients with history of rheumatic fever or rheumatic heart disease

Therapy Symptomatic relief Eradication of GAS Prophylaxis against future GAS infection to prevent recurrent disease Primary prevention most important: prompt diagnosis and treatment of group A streptococcal tonsillopharyngitis

Take Home Points Polyarthritis is typically the first manifestation but may present weeks after GAS tonsillopharyngitis Patients already treated with aspirin and NSAIDs may not meet criteria for ARF despite having the disorder Carditis is most severe manifestation and can be prevented by prompt treatment of GAS