INTRODUCTION Acute, recurrent, inflammatory disease, mainly of children(5-15 years) Typically occurring 1-5 weeks after group A beta- hemolytic streptococcal infection
ETIOPATHOGENESIS
LIPOTEICHOIC ACID M,T, R PROTEINS N-ACETYL GLUCOSAMINE HYALURONIC ACID
HISTOLOGY Fibrinoid degeneration in collagen of connective tissues. Aschoff nodules in the heart( pathognomonic ) Multinucleated giant cells surrounded by macrophages and T lymphocytes
CLINICAL FEATURES Acute RF: fever, anorexia, lethargy, joint pain 2-3 weeks after streptococcal pharyngitis JONES CRITERIA Positive culture – 25% (due to latent period) Increased serum ASO antibody titre Isolated chorea/ pancarditis – presumptive diagnosis, if other causes excluded. K/C/O previous RF or RHD – multiple minor criteria + evidence of previous group A streptococcal pharyngitis
JONES CRITERIA
Carditis (50-60%) Within 2 weeks of RF Pancarditis (90% - 3 years; 30% - adolescence) Associated with AR – 50%
ARTHRITIS (60-75%) Most common, early Acute, painful, asymmetric, migratory (flitting and fleeting) inflammation of large joints Knees, ankles, elbows, wrists Red, swollen, tender joints for 1-4 weeks. Pain typically responds to aspirin No residual deformity – JACCOD’S ARTHRITIS.
SKIN LESIONS ERYHTEMA MARGINATUM (<5%) Early Red at edges, fade in centre Trunk, proximal extremities (bathing suit distribution) Spares face Margins coalesce or overlap
SUBCUTANEOUS NODULES (5-7%) After 3 weeks of onset Small nodules 0.5-2 cm Painless, firm Extensor surface of bones, tendons Confirmatory Mostly associated with carditis
SYNDENHAM’S CHOREA (30%) St. Vitus Dance Late – 3 months after ARF More common in females Emotional liability Rapid, involuntary, purposeless, non-repetitive choreiform movements of hands, feet and face. Explosive, halting speech. Self limiting 25% - chronic rheumatic valve disease.
TAKE HOME Group A beta hemolytic streptococci Cross reactivity of host antistreptococcal Abs to cardiac antigens Aschoff nodules JONES CRITERIA ( 2 major or 1 major+2 minor) Rheumatic heart disease – mitral valve most commonly involved. Followed by aortic valve.