Rheumatic Fever and Rheumatic Heart Disease

1. Rheumatic Fever and Rheumatic Heart Disease DR.ABDUL GHAFFAR MEMON Associate Professor Cardiology LUMHS HYD/JAMSHORO

2. Learning Objectives: • To understand the pathogenesis of acute rheumatic fever and rheumatic heart disease • To appreciate the burden of disease • To recognize the features of a streptococcal sore throat • To know the treatment regimens of a streptococcal sore throat • To be aware of secondary prevention measures

3. Performance Objectives: • Examine the burden of disease within own communities • Timely recognition of a streptococcal sore throat with correct treatment • Institute secondary prevention programme • Join the global community fighting Rheumatic fever and rheumatic heart disease

4. What is the incidence of acute rheumatic fever and rheumatic heart disease?

5. ARF is a disease of child age group 6 – 15 yrs. ARF uncommon < 5 yrs. And recurrence very rare after 34 yrs. Pharyngitis 20 % bacterial Epidemiology 80 % viral 15% GABHS 5% other bact. .3 – 3 % ARF

6. Incidence of ARF: Population-based Studies

7. What is the prevalence of rheumatic heart disease?

9. agent host environ-ment -GABHS ->100 subtype of M protein Heart-myosin Heart valve- laminin Synovia- vimentin Skin-keratin Brain- lysoganglioside -Extent of immune response to pharyngitis -Genetic susceptibility -Prior history of RF Pathobiology -Over crowding -low socioeconomic status

10. Carapetis. Lancet 2005;366:155

11. What is the pathogenesis of acute rheumatic fever?

12. ACUTE RHEUMATIC FEVER • Autoimmune consequence of infection with Group A streptococcal infection • Results in a generalised inflammatory response affecting brains, joints, skin, subcutaneous tissues and the heart.

13. ACUTE RHEUMATIC FEVER • The clinical presentation can be vague and difficult to diagnose. • Currently the modified Duckett-Jones criteria form the basis of the diagnosis of the condition.

15. RHEUMATIC HEART DISEASE • Rheumatic Heart Disease is the permanent heart valve damage resulting from one or more attacks of ARF. • It is thought that 40-60% of patients with ARF will go on to developing RHD.

16. RHEUMATIC HEART DISEASE • The commonest valves affecting are the mitral and aortic, in that order. However all four valves can be affected.

17. RHEUMATIC HEART DISEASE • Sadly, RHD can go undetected with the result that patients present with debilitating heart failure. • At this stage surgery is the only possible treatment option.

18. RHEUMATIC HEART DISEASE • Patients living in poor countries have limited or no access to expensive heart surgery. • Prosthetic valves themselves are costly and associated with a not insignificant morbidity and mortality.

19. What are the clinical features of strep sore throat?

21. Hallmarks of STREP sore throat • Tender lymph nodes • Close contact with infected person • Scarlet fever rash • Excoriated nares( crusted lesions) in infants • Tonsillar exudates in older children • Abdominal pain • GOLD STANDARD: POSITIVE THROAT CULTURE

22. Hallmarks of VIRAL sore throat • Coryza: runny nose or mouth ulcers • Other family with COLD symptoms • Evidence of another viral infection • Itchy watery eyes • Hoarseness and cough: non-specific • Fever: not specific • Red Throat: not specific

24. Criteria 2002-03 WHO criteria for the diagnosis of RF and RHF Diagnostic Categories Primary episode of rheumatic fever Recurrent attack of RF in a pt. without stablished RHD Recurrent attack of RF in a pt. with stablished RHD Rheumatic chorea OR insidious onset rheumatic carditis. 2 major or 1 major plus 2 minor plus evidence of GAS Infection. Same as above 2minor manifestation plus evidence of GAS infection. No other major manifestations or evidence of GAS infection

25. Criteria Continue…. Diagnostic categories Chronic valve lesion of RHD (pts presenting for first time with pure MS or mixed mitral valve disease and/or aortic valve disease.) Do not require any other criteria to be diagnose as having RHD

26. Manifest in the 60 – 75% of pt. Painful, migratory, and limited to the major joint of extremities Inflammation in 1 joint lasts for 1-2 week and polyarthritis as whole resolve in 1 month or less. Tenderness out of proportion to other findings. JACCOUD Arthropathy can be occur. Arthritis can overlap carditis but both manifestation inversely related in severity. Very good response to salicylates. Arthritis

27. Manifest as valvulitis – (MR and/or AR) myocarditis or pericarditis or both. 40 – 60% result in RHD Soft blowing pansystolic murmur of MR is hallmark of carditis in RF. HF due to the MR not due to myocardial involvement. Pericarditis cause friction rub and sometime pleuritic chest pain. Myocardial inflammation can cause conduction defect and heart block. There is a linear relationship b/w severity of MR during the first episode of RF and subsequent RHD. Carditis

28. Continue…… In the setting of LV dysfunction or/and pericarditis myocarditis without valvular involvement path- ology is unlikely to rheumatic in origin.

29. Purposeless, rapid, involuntary, nonrepetitive, jerky dance like movements. Milk maid grip. Lizard tongue. Raising of hand above the head. 5 – 35% pt. of RF Prior to puberty F>>>M, after puberty no male involvement. Risk of developing subsequent RHD is 50%. Sydenham's chorea

30. Sydenham chorea No residual neurological deficit per se, psychiatric disturbances occur in a small no. of pts. Recurrence are common. PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infection) also a manifestation GABHS but not associated with other features of RF.

31. 5 – 13% of RF Begin 1-3 cm, pink to red, nonpruritic, macule or papule. Trunk and proximal limb but never on face. Central clearing in serpegious fashion Exacerbate by heat. Also can seen in sepsis, drug reaction, glomerulonephritis, JRA, Lyme disease. Occur in conjunction with milder form of carditis. May lasts for months or years. Erythema marginatum

32. Closer view of erythema marginatum in the same patient

33. 0 – 8% of pt. of RF External surface of elbow, knee, ankles, knuckles, scalp and spinal process. Firm, nontender, free from attachment of underlying skin. Strongly support the severity of carditis Resolve within weeks to 1 or 2 months. Not diagnostic for rheumatic fever can seen with other autoimmune disorder. Subcutaneous nodules

35. Recommended test in case of possible acute rheumatic fever.. CBC ESR CRP BLOOD CULTURE ECG X-RAY CHEST PA VIEW 2D-ECHO THROAT SWAB CULTURE FOR GAS (gold standard) ASO TITRE (rising) much specific for RF

36. What are the treatment regimens of streptococcal sore throat?

37. Treatment of ARF First line of symptomatic therapy is antiinflammatory agent ranging from salicylates to steroid. Naproxen can be alternative for Aspirin. Bed rest in carditis pts. Effective antibiotic treatment acutely (starting less than 10 days.)almost completely eliminates risk of the dis.

38. Treatment • Drug Name :Aspirin -- Begin administration immediately after diagnosis of RF. Initiation of therapy may mask manifestations of disease. • Adult Dose: 4-8 g/d PO divided q4-6h; maintain aspirin levels in 20-25 mg/dL range until all symptoms have resolved and APRs have returned to normal

39. Treatment • Drug Name: Prednisone -- Used in carditis and CHF. High-dose prednisone is administered for 2-3 wk, then tapered over 3 wk. IV corticosteroids are reserved for fulminant cases. • Adult Dose :40-60 mg PO qd for 2-3 wk initially, then discontinue by gradual taper over 3 wk

40. Treatment • Pediatric Dose :0.05-2 mg/kg PO qd for 2-3 wk initially, then discontinue by gradual taper over 3 wk • Contraindications • Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease

41. Treatment of GABHS Infection Benzathine 1.2 million U. One time Acutely penicillin G IM one Penicillin V 500 mg oral BD 10 days Amoxicillin 500 mg oral TDS 10 days Erythromycin 250 mg oral BD 10 days

42. Antibiotics • The roles for antibiotics are to (1)initially treat GAS pharyngitis, • (2) prevent recurrent streptococcal pharyngitis, RF, and RHD, and • (3) provide prophylaxis against bacterial endocarditis.

43. Treatment • Drug Name: Penicillin V -- DOC for treatment of GAS pharyngitis. • Do not use tetracycline and sulfonamides to treat GAS pharyngitis. For recurrent GAS pharyngitis, a second 10-d course of same antibiotic may be repeated.

44. Treatment • Drug Name: Benzathine /procaine penicillin -- Adult Dose: Eradication: 1.2 million U of benzathine penicillin G or a combination of 900,000 U of benzathine penicillin G with 300,000 U of procaine penicillin G IM as a single dose

45. Treatment • Erythromycin -- Used for patients who are allergic to penicillin. • Other options include clarithromycin, azithromycin, or a narrow-spectrum cephalosporin (ie, cephalexin). • As many as 15% of penicillin-allergic patients also are allergic to cephalosporins.

46. Treatment • Drug Name: Clarithromycin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin. • Adult Dose: 250-500 mg PO bid for 10d • Pediatric Dose: 7.5 mg/kg PO bid for 10 d

47. Treatment • Drug Name: Azithromycin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin. • Adult Dose :12 mg/kg (not to exceed 500 mg) PO qd for 5 d • Pediatric Dose :Administer as in adults

48. Treatment • Cephalexin -- Alternate antibiotic for treating GAS pharyngitis in patients allergic to penicillin. • Adult Dose: 250-500 mg PO qid for 10d • Pediatric Dose: 25-50 mg/kg/d PO divided qid for 10 d

49. Primordial prevention 1. Improvements in socioeconomic status. 2. Prevention of overcrowding. 3. Prevention of under nutrition and malnutrition.

50. Primary prevention