1. Streptococcal Infections: The Case of Acute Rheumatic Fever / Rheumatic Heart Disease Ahmed Mandil Prof of Epidemiology Family & Community Medicine Dept King Saud University

2. Headlines Streptococcal Infections Sore throat (streptococcal versus viral) Acute rheumatic fever Rheumatic heart disease Prevention and control November 12, 20152ARF/RHD

3. Types of Streptococcal Infections According to reaction on blood-agar plates: Αlpha-hemolytic group (Streptococcus viridans): produces hemolysis circled by a greenish ring surrounding the central colony Βeta-hemolytic group (Streptococcus pyogenes): produces a completely clear zone around the central colony November 12, 2015ARF/RHD3

4.  Upper respiratory infections (sore throat): acute pharyngitis or acute tonsillitis  Skin infections: impetigo, pyoderma  Other acute infections: scarlet fever, puerperal sepsis, septicemia, erysipelas, cellulitis, mastoiditis, otitis media, pneumonia, rarely: toxic shock syndrome  Non-suppurative complications: acute rheumatic fever (within 19 days on the average), acute glomerulo- nephritis (within 1-5 weeks on the average), rheumatic heart disease (days-weeks) Group A β-Hemolytic Streptococci: Clinical presentations November 12, 20154ARF/RHD

5. Group A β-Hemolytic Streptococci could be a precursor of two serious non-suppurative sequlae, namely: Post streptococcal glomerulonephritis Acute rheumatic fever and rheumatic heart disease Public Health Importance : November 12, 20155ARF/RHD

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

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9. Hallmarks of STREP sore throat Close contact with infected person Tender lymph nodes Excoriated nares (crusted lesions) in infants Tonsillar exudates in older children Scarlet fever rash Abdominal pain GOLD STANDARD: POSITIVE THROAT CULTURE November 12, 20159ARF/RHD

10. Hallmarks of VIRAL sore throat Other family member with COLD symptoms; evidence of other viral infection Coryza: runny nose or mouth ulcers Itchy watery eyes Hoarseness and cough: non-specific Fever: not specific Red Throat: not specific November 12, 201510ARF/RHD

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

12. Primary Prevention of Rheumatic Fever by treating sore throat Oral penicillin is less efficacious than Penicillin IMI Anaphylaxis is extremely unusual November 12, 201512ARF/RHD

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14. Is it cost-effective to administer penicillin for all cases of suspected strep sore throat? An overall protective effect for the use of penicillin against acute rheumatic fever of 80% with an NNT of 60 children per year to prevent 1 episode of rheumatic fever. Mild hypertension: have to treat 800 people per year to prevent 1 episode of stroke November 12, 201514ARF/RHD

15. Is it cost-effective to administer penicillin for all cases of suspected strep sore throat? The estimated cost of preventing one case of rheumatic fever by a single intramuscular injection of penicillin is US$46 Valve replacement surgery for 1 case of RHD is at least US$15, 000 Cardiac surgery in African nations: available in Egypt, South Africa, and Ghana November 12, 201515ARF/RHD

16. Acute Rheumatic Fever

17. Occurrence Children: 3-18 years, more in developing nations compared to developed Equal gender distribution Risk factors include: poor socio-economic conditions and access to healthcare Peak in colder months 2-6 weeks following GA-β hemolytic strep infection Sudden onset of fever, pallor, malaise November 12, 2015ARF/RHD17

18. Incidence of ARF: Population-based Studies November 12, 201518ARF/RHD

19. General Features Autoimmune consequence of infection with Group A streptococcal infection Results in a generalised inflammatory response affecting brains, joints, skin, subcutaneous tissues and the heart. Currently the modified Duckett-Jones criteria form the basis of the diagnosis of the condition. November 12, 201519ARF/RHD

20. Carapetis. Lancet 2005;366:155 November 12, 201520ARF/RHD

21. Jones’ Criteria Major criteria: arthritis; carditis; Sydenham’s chorea; erythema marginatum; subcutaneous nodules Minor criteria: fever; arthralgia; elevated C-reactive protein; Rising Erythrocyte Sedimentation Rate; prolonged PR-interval (on ECG examination) November 12, 2015ARF/RHD21

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23. Rheumatic Heart Disease

24. Overview - 1 Rheumatic Heart Disease is the permanent heart valve damage resulting from one or more attacks of ARF. It is estimated that 40-60% of patients with ARF will go on to developing RHD The commonest affected valves are the mitral and aortic, in that order. However all four valves could be affected. November 12, 201524ARF/RHD

25. Overview - 2 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. November 12, 201525ARF/RHD

26. Overview - 3 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. November 12, 201526ARF/RHD

27. In the Pacific Islander population of New Zealand the incidence rate of ARF is 80-100 per 100 000 compared to non-indigenous new Zealanders <10 per 100 000. In a recent systematic review of the incidence of first attack of rheumatic fever, a Maori community in New Zealand has a disturbingly high incidence of >80/100,000 per year. November 12, 201527ARF/RHD What is the incidence of acute rheumatic fever and rheumatic heart disease?

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29. Incidence of newly diagnosed RHD A prospective clinical registry captured data from new presentation of structural and functional valvular heart disease presenting to the department of cardiology in 2006/7. Of the 4005 de novo cases, 344 (8.6%) were diagnosed as having RHD. A significant proportion presented with complications and 22% subsequently underwent surgery. November 12, 201529ARF/RHD

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31. What is the prevalence of rheumatic heart disease? November 12, 201531ARF/RHD

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35. Prevention & Control

36. Basic principles 1 In some developing countries, remarkable progress has been made in terms of decreasing incidence of ARF In 1986 a comprehensive 10-year prevention programme was conducted in a Cuban province. This programme relied on comprehensive primary and secondary prevention of RF/RHD as well as awareness and education programmes November 12, 2015ARF/RHD36

37. Basic principles 2 The main content of the activities focused around early detection and treatment of sore throats and streptococcal pharyngitis The project also included primary and secondary prevention of RF/RHD, training of personnel, health education, dissemination of information, community involvement and epidemiological surveillance. November 12, 2015ARF/RHD37

38. Basic principles 3 There was a progressive decline in the occurrence and severity of acute RF and RHD, with a marked decrease in the prevalence of RHD in school children. A marked and progressive decline was also seen in the incidence and severity of ARF There was an even more marked reduction in recurrent attacks of RF as well as in the number and severity of patients requiring hospitalisation and surgical care. November 12, 2015ARF/RHD38

39. RHEUMATIC FEVER IS PREVENTABLE Costa Rica Cuba November 12, 201539ARF/RHD

40. Primary Prevention of Rheumatic Fever by treating sore throat Oral penicillin is less efficacious than Penicillin IMI Anaphylaxis is extremely unusual November 12, 201540ARF/RHD

41. Rheumatic Heart Disease: SECONDARY PREVENTION PICTURE TAKEN OUT FOR SPACE ISSUES

42. THIS IS TOO LATE November 12, 201542ARF/RHD

43. Secondary Prevention Stops sore throat, prevents recurrences of ARF and aids in regression of RHD Oral penicillin has been shown to be less effective than Penicillin IMI Anaphylaxis is extremely unusual November 12, 201543ARF/RHD

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45. During an episode of ARF, valve changes can be minor and are still able to regress. After recurrent episodes of ARF, thickening of subvalvar apparatus, chordal thickening and shortening and progression to permanent valve damage is evident. November 12, 201545ARF/RHD

46. Awareness ♦ Surveillance ♦ Advocacy ♦ Prevention Secondary prevention: Duration CATEGORYDURATION OF PROPHYLAXIS All persons with ARF with no or mild carditis MINIMUM 10 years after most recent episode or age 21 All persons with ARF and moderate carditis MINIMUM 10 years after most recent episode or age 35 All persons with ARF and severe carditis MINIMUM 10 years after most recent episode or age 35 and then specialist review for need to continue. Post surgical cases definitely lifelong. November 12, 201546ARF/RHD

47. Secondary prevention: specifics PENCILLIN Secondary prophylaxis also reduces the severity of RHD. It is associated with regression of heart disease in approximately 50-70% of those with good adherence over a decade and reduces mortality. Route: BPG is most effective when given as a deep intramuscular injection. November 12, 201547ARF/RHD

48. Secondary prevention: Adherence Use a 23-gauge needle- deeper is better Local pressure to area for 10 secs Warm syringe to room temperature First allow alcohol to dry or use ethylchloride spray. How can we reduce the pain associated with IM Penicillin ? November 12, 201548ARF/RHD

49. Secondary prevention: Adherence Deliver injection very slowly(over 2-3mins) Distraction techniques Good rapport with the case, is a significant aid to injection comfort, compliance and understanding. Use 0.5-1ml of 1% lignocaine. Reduces pain significantly and excellent for younger patients. November 12, 201549ARF/RHD

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51. Ensuring that patients understand their disease, are informed regarding their future and receive secondary prophylaxis EDUCATION Health education is critical at all levels Lack of parental awareness of the causes and consequences of ARF/RHD is a key contributor to poor adherence amongst children on long-term prophylaxis. November 12, 201551ARF/RHD

52. What is the role of a register- based programme?

53. Background In 1972, the WHO launched a register-based programme to combat ARF/RHD By 1990, registers had been established in 16 countries with over a million school-going children involved. However in 2001, the WHO ceased its funding to this global programme. Experience elsewhere however provides conclusive evidence of registers realising notable successes in reducing RF recurrence. November 12, 2015ARF/RHD53

54. Purposes Collect data on demographic profiles; Highlight deficiencies in service delivery Priority-based guidelines to evaluate and managepatients A register of cases of RF and RHD can be used to improve treatment adherence in order to prevent recurrent RF and the development of RHD, necessitating surgery. November 12, 2015ARF/RHD54

55. A.S.A.P. Programme for the Control of RHD in Africa: Focus areas for action Awareness raising: public, healthcare workers Surveillance: incidence, prevalence, temporal trends Advocacy: appropriate funding of the treatment and prevention programmes Prevention: application of existing knowledge in primary & secondary prevention November 12, 201555ARF/RHD

56. Conclusion Rheumatic heart disease is the only truly preventable chronic heart condition Primary prevention: Penicillin for suspected strep sore throat Secondary prevention Penicillin prophylaxis November 12, 201556ARF/RHD

57. References Heymann DL. Control of communicable diseases manual. Washington DC: American Public Health Association, 2008 Zühlke L. The prevention of rheumatic fever and rheumatic heart disease. Cape-Town: Red Cross War Memorial Childrens Hospital. http://www.who.int/cardiovascular_diseases/ resources/trs923/en http://www.pascar.co.za/C_ASAP.asp November 12, 2015ARF/RHD57

58. Thank you for your kind attention November 12, 201558ARF/RHD