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ACUTE RHEUMATIC FEVER

ETIOLOGY
Infection with group A beta hemolytic streptococcal infection Commonly results in pharyngitis and impetigo

Strain dependent
Strains like 1, 3 , 6, 8, 14 causes pharyngitis 2-3 weeks later the resulting autoimmunity results in rheumatic fever

ACUTE RHEUMATIC FEVER


INCIDENCE

Age group 5 to 15yrs Endemic Asia and Africa In 100 per 1,00,000 population this is the cause of acquired heart disease

PREDISPOSING FACTORS
Over crowding and poor living conditions Lower socio economic status
For every untreated case of streptococcal pharyngitis, there is 0.3% chance of developing a rheumatic fever Both sexes are equally affected.

AUTO IMMUNE THEORY


Trigered by an immune-mediated delayed response to infection with specific-strain of group A-streptococcus that posses antigens which may cross-react with cardiac myosin,

Antibodies produced against the Streptococcal antigen mediate inflammation - cardiac - joints - skin

Though the early manifestation is joint involvement the most damaging involvement is CARDIAC Hence the idiom, Rheumatic fever licks the joint, bites the heart

STREPTOCOCCI..
The M Serotypes 3,5,6,14,18,19 and 24 have strong association with CARDIAC Diseases Throat infection is commonly caused by serotypes 12,1,25,4 and 3. Strains of Streptococci leading to Rheumatic fever possess more of Streptolysin O than Streptolysin S.

PATHOLOGY
Pathological process consists of 1.Exudative stage.Fibrinoid necrosis of the connective tissue occur. 2.Proliferative stage - The hall mark of this stage is the formation of ASCHOFF Bodies - Composed of multinucleated giant cells surrounded by macrophages and T Lymphocytes.

CLINICAL FEATURES
Its a multisystem disorder typically following an episode of streptococcal pharyngitis. Presents withfever - anorexia - lethargy - joint pain

JONES CRITERIA
Major criteria Carditis Polyarthritis Chorea Erythema marginatum Subcutaneous nodules
Minor criteria Fever Arthralgia Previous rheumatic fever APR s Raised ESR
C-Reactive protein Leucocytosis
Prolonged

PR interval on

ECG.

Evidence of preceding streptococcal infection: Any one of the following is considered adequate evidence of infection: -Increased antistreptolysin O or other streptococcal antibodies

-Positive throat culture for Group A beta-hemolytic streptococci


-Positive rapid direct Group A strep carbohydrate antigen test -Recent scarlet fever DIAGNOSIS

Two major or one major and two minor criteria in addition to evidence of recent streptococcal infection

CARDITIS
Manifests asPANCARDITIS Involvement of Pericardium Rub can be present Myocardium Tachycardia Endocardium Valvulitis, (Murmurs - Carey Coombs) Development of cardiac enlargement or cardiac failure ECG changes of percarditis or myocarditis prolonged PR interval

ARTHRITIS
Migratory polyarthritis 40 60 % of patients Large joint involvement Knees, Elbows, Ankles and Wrists Joints are swollen,red and tender. As the inflamation recedes in one joint,the other becomes affected. Once the rheumatic process ends, the joints become NORMAL. No residual deformity

SYNDENHAMS CHOREA-[St.VITUS dance]10 20 % Occur later 3months after the episode of Acute rheumatic fever. Chorea alone is enough to diagnose acute rheumatic fever without satisfying other criteria. Involuntary movement Milk maid grip Jack in the Box tongue Hung up knee jerk Pronator sign

Erythema marginatum

5% of the patients Evanescent rash Pink rash with Slightly raised edges with central clearing Trunk and limbs

SUBCUTANEOUS NODULES
5 % of patients 0.3 3 cm in size Non tender Firm to hard nodule Freely mobile Palpable beneath the skin Particularly over tendons,joints and bony prominences. olecranon process occiput ulna shin of tibia Almost always associated with carditis

COURSE

AND

PROGRESS

Rheumatic fever tends to subside spontaneously in few weeks or months. Arthritis subsides but Carditis progresses. Recurrences and relapses occur invariably. Cardiac lesions worsens

INVESTIGATIONS
Throat Swabs-culture ESR,C-Reactive protein. ECG,---ECHO-Shows MR with dialatation of Mitral annulus and prolapse uf the Antr. Mitral leaflet.AR, Pericardial Effusion. SerodiagnosisASO Titre Evidence of Carditis-Chest x.ray;ECG,ECHO.

MANAGEMENT
Treatment of Acute attack Single dose of Inj. Benzathine Penicillin 1.2 Million units Oral Phenoxy methyl Penicillin 500 mg bd 10 days ALLERGIC to Penicillin

Erythromycin or Cephlosporin.

TRT..CONTD,.
Bed rest lessen joint pain reduces cardiac workload

Duration of Bed rest - Guided by fall of Temperature Leucocyte count,ESR. No sternuous excerise Cardiac failure has to be treated.

Aspirin 75 100 mg/kg body weight Example of a Therapeutic diagnosis 14 days later tapered Joint pain relief is dramatic
Corticosteroids Absolutely indicated in patients with carditis Prednisolone 1-2mg/kg/day in divided doses for 14 days , then tapered.

SECONDARY PREVENTION
Benzyl Penicillin 1.2 million units i.m. monthly Oral Phenoxymethyl Penicillin250mg bd daily Residual heart diseases -Prophylaxis life long Carditis without residual involvement No carditis

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