Definition: Acute, immunologically mediated multisystemic inflammatory disease following group A streptococcal pharyngitis.affecting joints, skin, heart and brain Occures in 3% of patients with group A streptococcal pharyngitis. Increase risk of reactivation with new pharyngeal infections

Diagrammatic structure of the group A beta hemolytic streptococcus Capsule Cell wall Protein antigens Group carbohydrate Peptidoglycan Cyto.membrane Cytoplasm …………………………………………… ……... Antigen of outer protein cell wall of GABHS induces antibody response in victim which result in autoimmune damage to heart valves, sub cutaneous tissue,tendons, joints & basal ganglia of brain

Pathogenesis of rheumatic fever Pathogenesis of rheumatic fever ARF is a hyper sensitivity reaction induced by group A streptococci. Antibodies directed against M proteins of streptococci cross-react with glycoproteins of heart,joints,skin and brain. Oncet of symptoms is 2-3 weeks after infection,and absence of bacteria in leasions.

Multinucleated giant cells, macrophages And T lymphpcytes seen only in the heart Found in myocardial biopsy Multinucleated giant cells, macrophages And T lymphpcytes seen only in the heart Found in myocardial biopsy

Clinical features Fever,anorexia,Lethargy,joint pain 2-3 weeks after an episode of streptococcal pharingitis Arthritis : migratory,asymmetrical affecting large joints {elbows,wrists, knees,ankles } Skin lesions: Erythema marginatum, subcutaneous nodules Cardites : usually pancarditis,cardiomegaly, murmurs, tachycardia,chest pain, Sydenhams chorea :occurs 3 m after acute RF Rare manifestations: pleurisy,pleural effusion,pneomonia Peak incidence 5-15 years

Migratory asymmetrical non -deforming Polyarthritis affects large joints Responds quickly to aspirin Occurs in 75% Joints are painful, red and warm For 1-7 days

Transient skin rash red macules over trunk proximal part of extremities pale center red margins which coalesce as snake like appearance, non pruritic, >10% 10%-15%, painless mobile over joints with normal skin color, small and transient 0.5-2mm in size, occures 3w after onset Associated with carditis

Dyspnoea, crdiomegaly, pericarditis, murmers, tachycardia, chest pain May be pancarditis incidence decrease with age 30% in adults ECG changes

Occures in 10% of patients, it is late manifestation of RF 3m Nonpurpossive, nonrepititive involantory movement of hands, face, or feet, more in females, Explosive speech..Emotional liability ………………. last 2-6m, spontaneous recovery is usual 25% go on to develop chronic RVD TR : halloperidole, phenobarbitone, Na-vlproate Carbimezapine St.Vitus`s dance

Major manifestations :*Cardtids *Polyarthritis * Chorea *Erythema marginatum *Subcutaneous nodules Minor manifestations :*Fever *Arthralgia *Previous RF Raised ESR or CR-protein * Leukocytosis * 1 st an 2 nd AV block Evidence of preceding streptococcal infection: *Raised ASO titer * Positive throat swab culture For diagnosis of RF: 2 Major or { 1 Major + 2 Minor } + Evidence of recent streptococcal Infection. Jones criteria for diagnosis of RF

Investigations: *Nonspecific : raised ESR, CRP, WBC *Evidence of preceeding strept. Infection : _ Throat swab culture for group A beta haemolytic strept. _ ASO titer > 200 u { adults }, and > 300 u { children} *Evidence of carditis : _Chest XR : cardiomegaly, pulmonary congestion _ ECG : 1st and 2nd AV block, ST, T, changes _ ECHO. : chamber dilatation, valve abnormalities

Treatment of acute rheumatic fever * Benzathine penicillin 1.2m units i.m to eliminate residual Strepto. infection * Bed rest and supportive measures : rest till symptoms improve * Aspirin mg /kg b.w in 6 doses for 3-4 ws * Corticosteroids in cases of carditis or severe arthritis prednisolone 1-2 mg/kg b.w in divided doses 2-3 ws tapering 20%/w * Secondary prevention _ Benzathine penicillin 1.2 million units i.m monthly or phenoxymethylpenicillin 250mg b.d or erythromycin until 21 year at least 5 years after last attack of acute RF

Chronic rheumatic carditis * occurs in 50% of those affected with RF with carditis * History of RF or chorea elicited only in 5o% of patients with chronic RVD * MV is affected in 90% of cases,AV next most frequent then TV and least frequently PV isolated MS occurs in 25% of all cases * Symptoms usually delayed for years or decades after acute RF * Predominant pathology is progressive fibrosis mainly affecting the valves causing thickening,deformity and calcification ending in varying degree of stenosis and /or regurgitation * Clinical features and complications depend on valves involved and include * Cardiac murmurs *Cardiac hypertrophy and dilatation *Congestive heart failure * Thromboembolic incidents *Infective endocarditis * Arrhythmias mainly AF