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 feverPathogenesis 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 featuresClinical 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 danceSt.Vitus`s dance

Major manifestations :*Cardtids *Polyarthritis * Chorea

*Erythema marginatum *Subcutaneous nodules

Minor manifestations :*Fever *Arthralgia *Previous RF Raised ESR or CR-protein * Leukocytosis * 1st an 2nd 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

IInvestigationsnvestigations::

*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 feverTreatment 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 60-120mg /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 carditisChronic 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