Rheumatic Fever

1. Rheumatic Fever Prof SC Brown M.MeD FCP (paed, cardio) DCH

2. How common is RHD? • 2 -3 % of infected GABHS  RF • WHO • 15.6 mill RHD • 300 000 of 0.5mill ARF > RHD p.a. • 233 000 deaths p.a. • Prevalence • Sub Saharan Africa • 5.7/1000 in children 5 – 14y • Asia • 2 – 3.5/100 • Developed countries • 0.5/1000 • Characteristics • Rare at young age (< 5% younger 5y) • F > m

3. Incidence ARF & RHD in Aboriginal Australians – Dpt of Health and community services, Northern Australia 400 35 RHD 2003 Incidence of ARF per 1000 200 Prevalence RHD per 1000 15 ARF 2002 < 5 5-14 15-24 25-34 > 34

4. Pathogenesis ARF

5. How do we make the diagnosis?

6. Natural progression of rheumatic fever Rx First contact with Streptococcus 5 – 15 yr Acute RF arthritis fever carditis skin I/T 2 – 3 w pharyngitis Prophylaxis! Jones criteria Jones criteria Relapse / acute on chronic Chronic RF MR, AR years MS, AS

7. MAJOR fleeting poliarhtritis carditis erythema marginatum subcut nodules Sydenhams chorea MINOR history of previous RF arthralgia fever PR prolongation - ECG LAB  WCC, ESR, CRP JONES CRITERIA: PLUS • EVIDENCE OF RECENT STREPTOCOCCAL INFECTION e.g. ASOT

8. Jones criteria

9. Drug Therapy anti-inflammatory antibiotics prevention

10. Treatment • not all tested in controlled trials • e.g. longterm bedrest for carditis • e.g. Pen for URTI • Not affect cardiac outcome after 1 y • anti-inflammatory • antibiotics

11. Anti-inflammatory • Salicylates • rapid resolution fever, arthritis, arhtralgia • evidence: • NOT be used carditis • do not decrease incidence of residual RHD • Dorfman (Pediatrics 1961): 12 week aspirin similar incidence of murmurs as controls > 1yr • meta analysis Cochrane 2003,CD003176) • No benefit over steroid or vice versa • indication • symptomatic Rx of fever, arthritis, arthralgia

12. Anti-inflammatory • steroids • reduce inflammatory response of ARF • esp. fever & acute phase reagents • little objective evidence • studies done before ECHO • meta-analyses no benefit over salisylates or placebo of RHD after 1 –10 y • most > 40y ago • not test newer corticosteroids

13. Anti-inflammatory: other • NSAIDS • Naproxen in 1 small trial • Immunoglobulins • IVIG in ARF – no diff > 1y • Chorea • Rx reserved moderate-severe symptoms • Valproic acid > carbamazepine or haloperidol • PROBLEM • need studies • natural progression of RHD • 60% regress after 10y

14. Prevention • oral • once daily amoxycillin • high dose amoxycillin • azithromycin • not recommended by US • intravenous • 3 - 4weekly • practical aspects • small gauge needles • 1% lignocaine or procaine penicillin • slow injection(3min) • direct pressure and warming of medication (room temp)

15. prophylaxis

16. prevention • treat carriers & children with sore throat • 1965 • possible reduction ARF 21% • cost US $12 pa per child or $65 000 per ARF prevented • New Zealand • school based sore throat diagnosis & treatment • no reduction in ARF incidence • programmes not practical or affordable • vaccine • multivalent vs M-serotype • unlikely < 2015

17. Subclinical Rheumatic carditis • “silent” mitral regurgutation  ECHO • MR jet > 1 • MR in 2 planes • velocity > 2,5m/s • chorea & arthritis: silent MR • abn valve – murmur 2w or 18mo -5y or MS • studies • trivial MR 45% normal children • ? higher in febrile patients • New Zealand study • Affect diagnosis of ARF in 10% of cases • summarise • controversial • problems with Echocardiography • WHO addition to Jones criteria  justified

18. role of ECHO • normal population • MR : 2,4 – 50% • AR : 0 – 33% • TR : 6,3 – 95% • PR : 21.9 – 92% • rheumatic fever • excludes nonrheumatic causes • e.g. prolapse, bicuspid aov • acute carditis • 25% nodules tip leaflets, disappear follow-up • classification of severity • 1+ to 4+ • no murmur • later develop RHD

19. Indications for referral to surgical centre (WHO) • symptoms have progressed beyond New York Heart Association(NYHA) Class II. Note: with aortic stenosis (AS), all symptomatic patients should be referred. • patients who are asymptomatic, or mildly symptomatic, with progressive left ventricular enlargement on clinical or radiological examination (>0.5 cm/year). • cardiac failure due to the valve lesion itself, rather than to an episode of rheumatic carditis. • Pulmonary hypertension, with physical signs and ECG evidence of changes in right ventricular hypertrophy, and chest X-ray evidence of pulmonary artery dilatation. • tricuspid regurgitation that complicates mitral valve disease. • development of atrial fibrillation. • thromboembolism. • endocarditis is suspected to contribute to cardiac decompensation.

20. surgery during acute phase n = 254 MR + HF age: 6 – 52y rheumatic activity : n = 76 higher incidence of early cx 5 ± 3y 47% acute mortality: 2.6% 5y mortality: 15% re-operation 27% conclusion “surgical valve repair during active carditis was associated with an acceptable survival rate, but reoperations were frequent” Circulation, 1994,90(5 Pt 2):II 167–174.

21. surgery during acute phase: conclusions • surgery can be safely performed during active carditis and, in of active carditis, may be preferable to the long-term use of corticosteroids. • myocardial inflammation plays no significant role in the clinical pathology of active carditis. • valve repair during active carditis may not constitute the best surgical option if there is macroscopic evidence of valvular inflammation, because valve repair is associated with significant reoperation rates.

22. conclusion • ? are we missing ARF • pathogenesis – answers • Jones criteria • challenge old ideas WHO Expert Consultation on Rheumatic Fever and Rheumatic Heart Disease (2001 : Geneva, Switzerland) Rheumatic fever and rheumatic heart disease

23. giving bad news gently