Rheumatic heard disease pediatrics.pptxk

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1. ACUTE RHEUMATIC FEVER AND RHEUMATIC HEART DISEASE DR ROGATUS DUWE (MMED 3 PCH) 1

2. 2 OBJ E C TIV E S To know about the epidemiology of the disease T o unde r st and the p a tho g enesis o f acute rheum a tic fever and rheumatic heart disease To know about the clinical features: cardiac & non-cardiac manifestations To learn about the laboratory studies of RHD To understand the principles of management

3. 3 ACUTE RHEUMATIC FEVER  Autoimmune consequence of infection with Group A streptococcal infection  Results in a generalised inflammatory response affecting brains, joints, skin, subcutaneous tissues, heart, respiratory system, vessels, serosal membranes, tendons and fascial sheaths  Clinical presentation can be vague and difficult to diagnose  Currently, the Jones criteria form the basis of the diagnosis of the condition

4. 4 Epidemiology Non suppurative complications of group A streptococcal pharyngitis Certain serotype of GAS (M type 1,3,5,6,18,24) A delayed immune response caused by antibody cross reactivity that can involve the heart, joints, skin and brain Latent period of 1-3 weeks Gram positive cocci rich in M protein is a virulence factor

5. Epidemiology Skin infection does not causes rheumatic fever or carditis because skin lipid cholesterol inhibit antigenicity Incidence of RF following streptococcal throat infection is 0.3%* Commonest age group 5- 15 yr First episode rare before 3 yr and after 30 yrs Male and female both equally affected *(IC M R su r v e y r es u l t 5 )

6. 6 Epidemiology cont …. Mitral valve disease and chorea are more common in girls Aortic valve involvement more common in boys Poor socioeconomic condition, unhygienic living conditions, overcrowded household predispose to streptococcal infections Common in tropics and subtropics Common in colder months

7. 7 Patho-physiology The cytotoxicity theory- GAS toxin produces enzyme streptolysin O The immune mediated theory- Immunological cross reactivity between the GAS components and mammalians tissue M protein M1,M5,M6, M19 share epitopes with human tropomyosin and myosin

8. 8 Patho-physiology Infection leads to rheumatic fever several weeks after the sore throat has resolved The organism spreads by direct contact with oral or respiratory secretions, and spread is enhanced by crowded living conditions Patients remain infected for weeks after symptomatic resolution of pharyngitis and may serve as a reservoir for infecting others

9. 9 Patho-physiology Inflammatory cell infiltration and proliferation of specific cells resulting in formation of ASHCOFF NODULES Resulting in- Pancarditis in heart Arthritis in joints Nodules in subcutaneous tissue Chorea

10. 10 Clinical presentation Modified Jones criteria (revised in 2015 ) provide guidelines for the diagnosis of rheumatic fever The Jones criteria require the presence of: 2 major Or 1 major and 2 minor criteria At least one essential criteria must be there in diagnosis of rheumatic fever A diagnosis of rheumatic heart disease is made after confirming antecedent rheumatic fever

11. 11 Major criteria Migratory poly arthritis Pancarditis Chorea Sub cutaneous nodules Erythema marginatum

12. 12 Minor crietria CLINICAL CRITERIA - Fever Arthralgia LABORATORY CRITERIA- Acute phase reactants ( CRP, ESR) Prolonged PR interval in ECG

13. 13 Essential Criteria Evidences of recent streptococcal infections Elevated ASLO titre Positive throat swab culture Rapid antigen test for group A streptococci

14. 14 ARTHRITIS Most common manifestation. (70%) Large joints (knee, elbow, ankle, wrist) Poly arthritis- succession or simultaneous Migratory in nature Swelling, heat, redness, severe pain, tenderness ,limitation of movement Responds drammatically with salicylates Subsides without residual deformity Lasts 1-5 weeks

15. CARDITIS Occurs in 50% of patients Tachycardia ( out of proportion to fever) Heart murmur of MR or AR or both Pancarditis ( pericarditis, myocarditis, endocarditis) 1 Endocardial- - MR or AR murmurs indicative of dilatation of valve with or without associated valvulitis Short mid-diastolic murmur (Carey-Coombs) may be present Changing quality of heart sounds Myocardial- Tachycardia even at rest Arrhythmias or ectopic beats Cardiomegaly- on physical exam, CXR or ECHO - Congestive cardiac failure – right or left sided 15

16. 16 CARDITIS Pericardial- Pericarditis Pericardial effusion ECG Changes- Changing contour of P waves Inversion of T waves Prolongation of PR interval Sign of CHF (gallop rhythm, cardiomegaly, distant heart sound) Maybe self limiting or may lead to slowly progressive valvular deformity Mitral valve attacked in 75% cases, aortic in 30% ( but rarely as the sole valve), tricuspid and pulmonary in < 5% cases

17. Thick valves, small vegetations Fish mouth mitral valve opening 17

18. 18 CHOR E A Sydenham’s chorea (St vitus’ dance) in 15% More common in prepubertal girls (8-12 yrs) Neuro psychiatric disorder Neurological - Choreic movement and hypotonia Psychiatric - emotional lability, hyperactivity, separation anxiety Begins with emotional lability replaced by choreic movement and then motor weakness

19. ERYTHEMA MARGINATUM In less than 10 % cases. Non-pruriti c annular erythematous rashes Trunk and inner proximal portion of extremities Never seen on face D isappears on exposure to cold Shape of rings with clear centers 19

20. 20 SUBCU T AN E OU S NO D UL E S 2- 10 % of cases Commonly in cases with recurrences Hard, painless, nonpruritic,freely movable swelling of 0.2 to 2 cm Extensor surface of both legs, small joints, scalp, spine Not transient, lasting for weeks Are recurrent Indistinguishable from rheumatoid nodules

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22. 22 Exception of jones criteria Chorea may occurs as the only manifestation of rheumatic fever Indolent carditis can be the only manifestation comes one month after the onset of RF Some time recurrences of rheumatic fever may not fulfill the Jones criteria

23. 23 Clinical course of disease Only carditis can cause permanent cardiac damage Sign of mild carditis disappears rapidly in weeks but severe carditis may last for 2 to 6 months Arthritis subsides within a few days to several week s Even without treatment does not causes permanent damage Chorea gradually subsides in 6-7 months or longer and does not causes permanent neurological sequele

24. 24 LABORATORY INVESTIGATIONS Rapid antigen detection test Throat culture Antistreptococcal antibodies The elevated level of antistreptococcal antibodies is useful, particularly in patients that present with chorea as the only diagnostic criterion 333 Todd Units Antibody titers should be checked at 2-week intervals in order to detect a rising titer

25. 25 LABORATORY INVESTIGATIONS Acute phase reactants (ESR,CRP) - Both tests have a high sensitivity but low specificity for rheumatic fever

26. 26 OTHER INVESTIGATIONS CHEST X-RAYS : Cardiomegaly Pulmonary congestion ECHO : Annular dilatation Elongation of the chordae to the anterior leaflet A postero laterally directed mitral regurgitation jet The left ventricle is frequently dilated in association with a normal or increased fractional shortening

27. 27 OTHER INVESTIGATIONS CONT…. ECG: Sinus tachycardia Sinus bradycardia First-degree atrioventricular (AV) block (prolongation of the PR interval) ST segment elevation may be present and is marked most in lead II, III, aVF, and V 4 -V 6

28. 28 OTHER INVESTIGATIONS Heart catheterization- In acute rheumatic heart disease, this procedure is not indicated With chronic disease , heart catheterization has been performed to evaluate mitral and aortic valve disease and to balloon stenotic mitral valves

29. 29 HISTOLOGIC FINDINGS Aschoff bodies: Perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophages Found in the pericardium, perivascular regions of the myocardium, and endocardium

30. chronic rheumatic heart disease (in adults) 30 TREATMENT AND MANAGEMENT  Therapy is directed towards: Eliminating the group A streptococcal pharyngitis (if still present) Suppressing inflammation from the autoimmune response Providing supportive treatment for congestive heart failure  Following the resolution of the acute episode, subsequent therapy is directed towards : Preventing recurrent rheumatic heart disease (in children) Monitoring for the complications and sequelae of

31. Drugs for primary prophylaxis of acute rheumatic fever Drugs Doses Sore throat treatment BENZATHINE PENIC I LLI N G De e p I M a f t er Sensitivity test 1.2 million unit (>27 kg) 0.6 million unit (<27 kg) Single dose PENICILLIN V (oral) CHILDREN – 250 mg QID ADULTS – 500 mg TID 10 days AZITHROMYCIN (oral) 12.5 mg/kg/day Once daily 5 days CEPHALEXIN (oral) 15-20 mg/kg/dose BD 10 days 32

32. 32 Prevention of rheumatic fever Primary prevention- 10 days course of penicillin therapy for streptococcal pharyngitis Patient sensitive to penicillin should advise erythromycin 20-40 mg/kg in two divide dose Secondary prophylaxis- Patient with documented history of rheumatic fever, isolated chorea, those without evidence of rheumatic heart disease must receive prophylaxis

33. 33 SECONDARY PROPHYLAXIS Benzathine penicillin G 1.2 million units given intra muscularly every 21 to 28 days after sensitivity testing Alternative method if any reaction to penicillin: Oral penicillin V 250 mg twice daily Oral sulfadiazine 1 gm once daily Oral sulfisoxazole 0.5 gm once daily Oral erythromycin 250 mg BD

34. Duration of prophylaxis for rheumatic fever Category Duration RHEUMATIC FEVER WITHOUT CARDITIS 5 yr or until age 21 yr Whichever is longer RF WITH CARDITIS BUT WITHOUT RESIDUAL HEART DISEASE (NO VALVULAR HEART DISEASE) 10 yr or well into adulthood Whichever is longer RF WITH CARDITIS AND RESIDUAL HEART DISEASE (PERSISTENT VALVULAR HEART DISEASE) At least 10 yr since last episode and last until age 40 yr Some time life long prophylaxis 35

35. 35 Management of rheumatic fever Bed rest: Duration depends on type and severity of manifestation One week for isolated arthritis Several weeks for severe carditis Full activity is allowed when ESR becomes normal Anti -inflammatory drugs : Mild to moderate carditis- Aspirin 90-120 mg/kg/day in 4-6 divided doses for 4-6 weeks then tapering of 75 mg /kg /day in next 2 weeks

36. 36 Management of rheumatic fever Severe carditis- Add steroid prednisone 2 mg /kg/day in four divided doses for 2-6 weeks ( If weight > 20 kg,dose of steroid 60mg/day for 3 weeks then 50mg/day for one week then 40 mg/day for next week, then reduce dose 5mg per week If weight <20 kg,dose of steroid 40mg/day for 2 weeks then reduce by 5 mg/week)

37. 37 Management of rheumatic fever Arthritis- Aspirin therapy for 2 weeks then gradually tapering over 2-3 weeks Treatment of CHF- Complete bed rest, oxygen Prednisone for severe carditis of recent onset Digoxin or furosemide if indicated

38. Arthr i tis alone Mild ca r ditis Mode r a t e carditis Severe ca r ditis Bed rest 1-2 weeks 3-4 weeks 4-6 weeks As long as congestive heart failure present Indoor Ambul a tio n 1-2 weeks 3-4 weeks 4-6 weeks 2-4 months Prednisone 2-6 weeks aspirin 1-2 weeks 3-4 weeks 6-8 weeks 2-4 months 39

39. 39 Management of rheumatic fever Management of chorea- Usually self limiting Reduce physical and mental stress Anti inflammatory agents are not needed in patient with isolated chorea For severe cases: ( Phenobarbitone, haloperidol , Valproic acid)

40. 40 PROGNOSIS OF RHEUMATIC FEVER Presence or absence of permanent cardiac damage Cardiac status at the start of treatment Recurrence of rheumatic fever Regression of heart disease

41. 41 RHEUMATIC HEART DISEASE Results from single or repeated attacks of RF Rigidity and deformity of valves resulting in stenosis or incompetence or both Mitral valve alone in 50% Mitral + Aortic in 25% Pure aortic uncommon History of RF obtained in 60%

42. 42 Treatment for patients following rheumatic heart disease (RHD) Preventive and prophylactic therapy is indicated after rheumatic fever and acute rheumatic heart disease to prevent further damage to valves Patients with rheumatic heart disease and valve damage require a single dose of antibiotics 1 hour before surgical and dental procedures to help prevent bacterial endocarditis Patients who had rheumatic fever without valve damage do not need endocarditis prophylaxis

43. 43 Surgical Care Indication: Heart failure persists or worsens after aggressive medical therapy for acute RHD, surgery to decrease valve insufficiency may be life- saving 40% of patients with acute rheumatic heart disease subsequently develop mitral stenosis as adults  Procedures: - Mitral valvulotomy - Percutaneous balloon valvuloplasty - Mitral valve replacement

44. Reference: UpToDate

45. 45 THANKS FO R LISTENING