• Definition: A multisystem disease resulting from an autoimmune rxn to infection with group A streptococcus.• RHD is the most common cause of heart disease in children in developing countries.
Epidemology• World wide 15-19million people affected.• Disease of children (5-14yrs)• Rare in adults especially >30yrs• Recurrence is more common in adults.• RHD peaks 25-40yrs
pathogenesis• Caused by infection of URTI with Group A streptococci .• Following this infection an autoimmune reaction results which lead to damage to human tissues as a result of cross reactivity between epitopes of the bacteria and host. (molecular mimicry)
Carditis• 60% of patients with ARF progress to RHD• Endocardium,myocardium,pericardium• Valvular involvement is the hallmark.• Mitral valve is almost alwayes involved• MR>MR+AR>TR• Early in the course regurgitation is common then progresses to stenosis.
migratory polyarthritis• Must be – Arthritic( swollen,hot,red,tender) – Very painful – > one joint – Migratory over hours• Large joint (knee,ankle,hip,elbow)• Highly responsive to salicylates and NSAIDS
Chorea• AKA sydenhams chorea• Usually presents alone.• Choreiform movements could involve head (dart like movt of the tongue) upper limb.• Eventually resolves completely within 6wks.
TREATMENT• Nothing to prevent the development or severity of RHD.• Rx is symptomatic• Rx of heart failure• Penicillin G 1.2mill IM stat or pen V 500mg po BID for 10 days for all.• ASA or NSAIDS for arthritis• Steroids for sever carditis• Carbamazepin or valproate for chorea
prevention• Primary prevention – Reduction of major risk factors (over crowding,unhygenic conditions) – Complete and timely Rx of URTI single dose IM cry.pen or po for 10days Erythromycin for pen allergy.
Secondary prevention• To prevent rheumatic recurrence• Benz.pen 1.2 mill units IM every 4wks• Or pen V 250mg 2x /wk po• Erythromycin 2x/day• Duration of RX 1- no carditis -5yrs after 1st attack or upto 18yrs 2-with mild carditis-10yrs after 1st attack or upto 25yrs 3-severe valvular disease or valve surgery-lifelong