DR G SWARNA LATHA DEVI M D PRO

DR. G. SWARNA LATHA DEVI M. D PRO. OF MEDICINE

Etiology Acute rheumatic fever is a systemic disease of childhood, often recurrent that follows group A beta hemolytic streptococcal infection n It is a delayed non-suppurative sequelae to URTI with GABH streptococci. n It is a diffuse inflammatory disease of connective tissue, primarily involving heart, blood vessels, joints, subcut. tissue and CNS n 2

Epidemiology n Ages 5 -15 yrs are most susceptible Rare <3 yrs Girls>boys Common in 3 rd world countries n Environmental factors-- over crowding, n n n poor sanitation, poverty, n Incidence more during RAINfall , winter & early spring 3

Pathogenesis n n Delayed immune response to infection with group. A beta hemolytic streptococci. After a latent period of 1 -3 weeks, antibody induced immunological damage occur to heart valves, joints, subcutaneous tissue & basal ganglia of brain 4

Group A Beta Hemolytic Streptococcus n Strains that produces rheumatic fever M types l, 3, 5, 6, 18 & 24 n Pharyngitis- produced by GABHS can lead to- acute rheumatic fever , rheumatic heart disease & post strept. Glomerulonephritis n Skin infection- produced by GABHS leads to post streptococcal glomerulo nephritis only. It will not result in Rh. Fever or carditis as skin lipid cholesterol inhibit antigenicity 5

Diagrammatic structure of the group A beta hemolytic streptococcus Antigen of outer protein cell wall of GABHS Cell wall induces antibody Protein antigens response in victim which Group carbohydrate result in autoimmune Peptidoglycan damage to heart valves, Cyto. membrane sub cutaneous tissue, tendons, Cytoplasm joints & basal ganglia of brain Capsule ……………………… ……. . . 6

Pathologic Lesions n Fibrinoid degeneration of connective tissue, inflammatory edema, inflammatory cell infiltration & proliferation of specific cells resulting in formation of Ashcoff nodules, resulting in-Pancarditis in the heart -Arthritis in the joints -Ashcoff nodules in the subcutaneous tissue -Basal gangliar lesions resulting in CHOREA 7

ACUTE RHEUMATIC FEVER Redness & swelling of throat & tonsils; Beefy, swollen, red uvula; Soft palate petechiae (“doughnut lesions”) Sore throat: fever, white draining patches on the throat & swollen or tender lymph glands in the neck Tonsillopharyngeal erythema & exudates

Histology of Myocardium in Rheumatic Carditis (200 X) 9

Clinical Features - ACCNE ARTHRITIS Flitting & fleeting migratory polyarthritis, involving major joints n Commonly involved jointsknee, ankle, elbow & wrist n Occur in 80%, involved joints are exquisitely tender n In children below 5 yrs arthritis usually mild but carditis more prominent n Arthritis do not progress to chronic disease n 11

Clinical Features (Contd) 2. Carditis Manifest as pancarditis(endocarditis, myocarditis and pericarditis), occur in 4050% of cases n Carditis is the only manifestation of rheumatic fever that leaves a sequelae & permanent damage to the organ n Valvulitis occur in acute phase n Chronic phase- fibrosis, calcification & stenosis of heart valves(fishmouth valves) n 12

Rheumatic heart disease. Abnormal mitral valve. Thick, fused chordae 13

Clinical Features (Contd) 3. Sydenham Chorea Occur in 5 -10% of cases n Mainly in girls of 1 -15 yrs age n May appear even 6/12 after the attack of rheumatic fever n Clinically manifest as-clumsiness, deterioration of hand writing, emotional instability , grimacing movements of face. n Clinical signs- pronator sign, jack in the box sign , milking sign of hands n 14

Clinical Features (Contd) 4. Erythema Marginatum Occur in <5%. n Unique, transient, serpiginous-looking lesions of 1 -2 inches in size n Pale center with red irregular margin n More on trunks & limbs & non-itchy n Worsens with application of heat n Often associated with chronic carditis n 18

ERYTHEMA MARGINATUM

Clinical Features (Contd) 5. Subcutaneous nodules Occur in 10% n Painless, pea-sized, palpable nodules n Mainly over extensor surfaces of joints, spine, scapulae & scalp n Associated with strong seropositivity n Always associated with severe carditis n 21

SUBCUTANEOUS NODULES

Recommendations of the American Heart Association 24

Clinical Features (Contd) Other features (Minor features) Fever-(upto 101 degree F) n Arthralgia n Pallor n Anorexia n Loss of weight n 26

Laboratory Findings High ESR n Anemia, leucocytosis n Elevated C-reactive protien n ASO titre >200 Todd units. (Peak value attained at 3 weeks, then comes down to normal by 6 weeks) n Anti-DNAse B test n Throat culture-GABHstreptococci n 27

Laboratory Findings (Contd) ECG- prolonged PR interval, 2 nd or 3 rd degree blocks, ST depression, T inversion n 2 D Echo cardiography- valve edema, mitral regurgitation, LA & LV dilatation, pericardial effusion, decreased contractility n 28

primarily prolonged PR interval

Cardiomegaly

Diagnosis Rheumatic fever is mainly a clinical diagnosis n No single diagnostic sign or specific laboratory test available for diagnosis n n Diagnosis based on MODIFIED JONES CRITERIA 31

Differential Diagnosis Juvenile rheumatiod arthritis n Septic arthritis n Sickle-cell arthropathy n n Myocarditis n Leukemia 32

Treatment Step I - primary prevention (eradication of streptococci) n Step II - anti inflammatory treatment (aspirin, steroids) n Step III- supportive management & management of complications n Step IV- secondary prevention (prevention of recurrent attacks) n 33

STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis) Agent Dose Benzathine penicillin G Mode 600 000 U for patients Intramuscular 27 kg (60 lb) 1 200 000 U for patients >27 kg Duration Once or Penicillin V Children: 250 mg 2 -3 times daily Oral (phenoxymethyl penicillin) Adolescents and adults: 500 mg 2 -3 times daily 10 d For individuals allergic to penicillin Erythromycin: Estolate 20 -40 mg/kg/d 2 -4 times daily (maximum 1 g/d) Oral 10 d or Ethylsuccinate 40 mg/kg/d 2 -4 times daily (maximum 1 g/d) Recommendations of American Heart Association Dr. Said Alavi 34

Step II: Anti inflammatory treatment Clinical condition Drugs 35

3. Step III: Supportive management & management of complications Bed rest n Treatment of congestive cardiac failure: -digitalis, diuretics n Treatment of chorea: -diazepam or haloperidol n Rest to joints & supportive splinting n 36

STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks) Agent Dose Benzathine penicillin G Mode 1 200 000 U every 3 weeks* Intramuscular or Penicillin V 250 mg twice daily Oral or Sulfadiazine 0. 5 g once daily for patients 27 kg (60 lb Oral 1. 0 g once daily for patients >27 kg (60 lb) For individuals allergic to penicillin and sulfadiazine Erythromycin 250 mg twice daily Oral *In high-risk situations, administration every 3 weeks is justified and recommended Recommendations of. Dr. Said American Heart Association Alavi 37

Duration of Secondary Rheumatic Fever Prophylaxis Category Duration Rheumatic fever with carditis and residual heart disease (persistent valvar disease*) At least 10 y since last episode and at least until age 40 y, sometimes lifelong prophylaxis Rheumatic fever with carditis but no residual heart disease valvar disease*) 10 y or well into adulthood, whichever is longer (no Rheumatic fever without carditis 5 y or until age 21 y, whichever is longer *Clinical or echocardiographic Recommendations of evidence. American Heart Association 38

Prognosis Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines n Good prognosis for older age group & if no carditis during the initial attack n Bad prognosis for younger children & those with carditis with valvar lesions n 39

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