Presentation Overview1. ARF: Clinical aspects • What is it? • Who gets it? • Diagnosis • Management2. RHD: Clinical aspects • Why all the fuss? • Secondary prophylaxis • Other management issues3. RHD Control programs4. Clinical scenarios5. Questions
ARF & RHDEverything you needto know!www.heartfoundation.org.au
1. Acute Rheumatic Fever (ARF)• What is it? • Auto-immune inflammatory response to infection with group A streptococcus (GAS = S. pyogenes) • Origin of GAS infection may be throat or skin • ARF is a multisystem disease affecting: – Joints (arthritis) – Heart (carditis) – Brain (chorea) – Skin (erythema marginatum) – Subcutaneous tissues (subcutaneous nodules)
Acute Rheumatic Fever• Who gets it? • Young people: peak incidence age 5-15 years • Previously common throughout the world • Now essentially confined to developing countries ... except Australia and NZ: • Very high rates in our remote Indigenous populations NHF ‘High risk group’ NT Indigenous* ARF incidence >30 ~250 (per 100,000 5-14yo, per yr) RHD prevalence >2 ~20 (per 1,000 all age) *Parnaby, M. G. and J. R. Carapetis (2010). "Rheumatic fever in Indigenous Australian children." J Paediatr Child Health 46(9): 527-533.
More sobering statistics:• ATSI people vs. other Australians: • 10 times more likely to get ARF • 10 times more likely to have RHD • 8 times more likely to be hospitalised with ARF/RHD • 20 times more likely to die from ARF/RHD • Mean age of death 39 years...
ARF: Diagnosis• Could it be ARF??? • Diagnosis can be difficult – Non-specific symptoms – No single diagnostic test – Many clinicians have never seen a case • Traditionally diagnosed using Jones Criteria • NHF Australia published modified criteria in 2006
2006 Australian guidelinesDiagnosis of Acute Rheumatic Fever in High Risk GroupsMajor criteria • Carditis* (including subclinical) • Polyarthritis OR Monoarthritis OR Polyarthralgia • Chorea# • Erythema marginatum • Subcutaneous nodulesMinor criteria • Fever (≥38ºC) • ESR ≥ 30mm/hr OR CRP ≥ 20 mg/L • Prolonged PR interval on ECG§Evidence of Group A Streptococcal • GAS cultured from throat swab(GAS) infection • ASOT OR Anti-DNaseB* Including subclinical evidence of RHD on echocardiogram# Rheumatic (Sydenham’s) chorea is diagnostic of ARF if other causes have been excluded. It does not require any other criteria or evidence of GAS infection.§ If carditis is present as a major manifestation, prolonged PR interval cannot be considered as an additional minor manifestation in the same person.
2006 Australian guidelines Diagnostic criteria for initial episode of ARF 2 major criteria OR 1 major and 2 minor PLUS Evidence of preceding Group A Streptococcal infection Diagnostic criteria for recurrent episode of ARF 2 major criteria OR 1 major and 2 minor OR 3 minor PLUS Evidence of preceding Group A Streptococcal infection
ARF: Diagnosis• Think ARF if: • Painful, swollen joint(s); most common presentation – May only be 1 joint – Not necessarily ‘migratory’ or ‘fleeting’ – Commonly knee, ankle, wrist, elbow) • Fever, lethargy • Abnormal movements (chorea) • New heart murmur • Heart failure (shortness of breath, fast pulse etc) • Rash or nodules (rare) • Past history of ARF/RHD (recurrence is common and causes the most heart damage)
ARF: Management• Management in clinic • Check vital signs • Listen to heart & lungs • Do ECG • Treat pain with paracetamol • Try to avoid NSAIDs or aspirin (can confuse diagnosis) • Can give IM Benzathine penicillin, but not urgent • Send to hospital – Urgently if heart failure – Within 1 day if arthritis – Within 1 week if chorea only
ARF: Management• If delay in transferring to hospital: • Liaise with DMO/specialist • Ensure adequate pain relief • Give IM Benzathine penicillin • Do investigations: – Throat swab for MCS – Streptococcal serology (ASOT, AntiDNAseB) – FBE – CRP, ESR – Blood culture
Acute Rheumatic Fever• Management in hospital • All patients should receive IM Benzathine penicillin • Other treatment is symptomatic: – Arthritis: aspirin – Carditis: bed rest/diuretics/ACEi/steroids/digoxin – Chorea: nil/carbamazepine/valproic acid • All patients should have an echocardiogram • All patients should be seen by RHD register staff • All patients should be discharged on prophylactic penicillin
Why all the fuss?? * Sickness builds up and heart valves leak.Rhematic fever comes back. Heart becomes weak. Often many times. Bad health and early death.
2. Rheumatic Heart Disease (RHD)• The only long-term complication of ARF• Damage to heart valves results from the inflammation of ARF• Predominantly affects mitral and aortic valves• Can be subclinical (no symptoms or murmur)• Can be asymptomatic (murmur but no symptoms)• Can lead to heart failure• Valve damage worsens with recurrent episodes of ARF• Secondary prophylaxis with penicillin is currently the only proven intervention that improves prognosis (if properly delivered)
RHD: Secondary prophylaxis• A word about Penicillin: • Many different types! Check preparation instructions • Need IM Benzathine penicillin G – Delivers low dose penicillin over 3-4 weeks – <20kg: 1ml (450mg or 600,000IU) – >20kg: 2ml (900mg or 1.2 million IU) • It hurts! See CARPA for delivery techniques & suggestions • How often? – Every 4 weeks; recurrence can occur if 1 dose missed • How long? – Minimum 10 years or until age 21 – Longer if valve damage, lifelong if valve surgery – Specialist to decide when to stop
RHD: Other management issues• Alternative 2° prophylaxis: • Daily oral penicillin V (adherence very problematic) • If truly penicillin allergic: – Penicillin desensitisation in hospital – Daily oral erythromycin• Medical review: • Depends on severity of RHD • All patients categorised by Priority (1=severe, 3=mild) • Minimum for children <18 yo: – Paediatric/physician review every 12 months – Dental review every 12 months – Echocardiogram every 2 years • Pneumococcal & flu immunisations important
Current RHD control options• Primordial prevention of ARF – Social determinants: housing, education, access to services• Primary prevention of ARF – Penicillin treatment for sore throat and skin sores – No vaccine yet• Secondary prevention of recurrent ARF & RHD – Penicillin prophylaxis for all known cases of ARF/RHD – Only intervention proven to be effective if properly delivered• Tertiary management of RHD • Medical treatment of cardiac failure • Cardiac surgery
RHD Control Programs• Aims: • Improve uptake and adherence to 2° prophylaxis • Improve clinical care and follow-up • Provide education and training for: – Patients and families – Health Care providers • Promote primary prevention (eg tonsillitis/skin sore treatment) • Identify and register new cases of ARF/RHD • Use data to: – Monitor patient outcomes – Evaluate and improve program strategies
RHD Control Programs• NT RHD Control Program: • Established 1997 in Top End, 2000 in Central Australia • Staff: 1 coordinator, 3 Darwin, 1 Katherine, 2 Alice Springs • Web-based register: – Anyone can apply for access – Useful clinical information (last review, last echo etc) – Generates recall lists• RHD Australia: • Established 2009; Federal funding • Aims to establish similar systems in WA and Qld • Will ultimately be national data source • Website has links to useful resources • www.rhdaustralia.org.au
Clinical scenario 1• 8 yo boy in remote Indigenous community• Presents to clinic with a sore ankle & fever• Fell of bike the day before• O/E: • Miserable but not unwell. Limping • Temp 38.1, other vital signs normal • No obvious murmur • Left ankle: swollen and hot, not red • Skin sores++ on left leg• What is the differential diagnosis• What do you do?
Clinical scenario 2• 8 yo girl in remote Indigenous community• Presents to clinic with SOB, cough & fever• Had skin sores ‘a while ago’• FHx of RHD• O/E: • Unwell, resp distress • PR 120, RR 50, Temp 38 • Bilateral wheeze/creps • ?systolic murmur• What is the differential diagnosis• What do you do?
Clinical scenario 3• 14 yo girl in remote Indigenous community• Presents to clinic with ‘can’t sit still anymore’• No history of skin sores or sore throat• O/E: • Not unwell; vital signs normal • No murmur • Strange movements of arm and face • Speech difficult to understand • Intermittently laughs for no reason• What is the differential diagnosis• What do you do?