Diagnosis of ARF in children

1. Diagnosis of ARF in children

2. Speakers Alan RubenFRACP, FAFPHM Paediatrician and Public Health Physician, ApunipimaCape York Health Council, Cairns and Hinterland Health Service District, Queensland Health. Alan is a paediatrician and public health physician who has worked in Aboriginal health for over 20 years. Ben Reeves MBBS, FRACPPaediatric cardiologist, Cairns and Hinterland Health Service District, Queensland Health. Ben is a paediatric cardiologist based in Cairns, providing outreach paediatric cardiology services to Cape York and the Torres Strait.

3. Learning objectives • Appreciate the pathway to ARF and then RHD • Recognize who is at risk for ARF/RHD • Understand the Jones criteria used for diagnosis • Present the recommended investigations • Outline current management guidelines

4. Take home messages • Incidence in Aboriginal Australians and Torres Strait Islander people amongst the highest in the world • Predominantly affects children aged 5 to 15 • Largely affects disadvantaged populations • High index of suspicion in high risk populations • Diagnosis needs clinical criteria and investigation results • Diagnosis often requires hospital admission

5. Abbreviations

6. More information – Guidelines www.rhdaustralia.org.au

7. More information – Quick reference www.rhdaustralia.org.au

8. More information – other modules

9. ARF: some basics • 3-6% of any population susceptible • Incidence and prevalence in females >males • ARF/RHD can run in families • Specific genetic markers have been identified • There is no racial predisposition

10. Australian setting • Amongst the highest rates in the world • ARF commonest in remote and disadvantaged areas • Some Australian medical staff unfamiliar with ARF

11. Environment

12. Risk factors Established clear link with poverty household overcrowding poor sanitation housing quality and appropriateness educational disadvantage Limited access to health services variability of health infrastructure and follow up Geographically remote

13. Acute rheumatic fever – ARF Chorea Carditis GAS pharyngitis Exaggerated immune response Arthritis Fever

14. * • ARF progression Valve damage is cumulative and silent Rheumatic heart disease (RHD) Cardiac failure, early death ARF recurs - often many times

15. Jones criteria

16. Diagnosis and GAS • Definite initial or recurrent ARF diagnosis requires: • 2 major plus evidence GAS infection • 1 major plus 2 minor plus evidence of GAS infection • Throat swab • ASOT • Anti DNAseB • No other probable diagnosis

17. Majormanifestations

18. Majormanifestations • High risk groups • Low Risk groups • Polyarthritis • Polyarthritis or aseptic mono-arthritis or polyarthralgia • Carditis • Carditis(including subclinical evidence of rheumatic valvulitis on echocardiogram) • Chorea • Erythema marginatum • Chorea • Subcutaneous nodules • Erythema marginatum • Subcutaneous nodules

19. Arthritis • Monoarthritis present in 17% of ARF presentations • Migratory asymmetric polyarthritis • Affects peripheral large joints • Often intense pain – will not tolerate passive movement • Limited duration: 2 days to 3 weeks • Dramatic response to salicylates • rapid response assists diagnosis

20. In high risk populations: • aseptic monoarthritiscan be a major manifestation • monoarthritisoften associated with carditis • if joint aspirate sterile, prior to treatment for septic arthritis, investigate for ARF Can a monarthritis be ARF?

21. A major criteria ONLY in high risk populations: • Multiple painful joints • Can be migratory • Unlike arthritis lacks: • Effusions • Heat • Morning stiffness Polyarthralgia

22. Carditis • Can involve all layers of the heart • Pericardium – can cause effusions • Myocardium – affects heart function and conduction • Endocardium – the classic valve lesions • MR then AR most common lesions • Right sided valves rarely involved • Stenosis is a late finding

23. Early echocardiography essential • repeated at 2 to 6 weeks • Chest x-ray • Electrocardiogram Carditis: investigations

24. Carditis: treatment • Often requiresinpatient bed restand care if : • moderate/severe carditis suspected by clinical findings • Consider steroids for severe carditis • If signs of heart failure or cardiomegaly • consider diuretics and ACE inhibitors

25. Sydenham’s chorea • Rapid, uncoordinated jerking movements • Primarily the face, feet and hands • Female to maleratio of 2:1 • Occurs up to 6 months after acute infection • Mostly children, 5 to 13 years • “Milkmaids” sign • Tongue fasciculations • Emotional lability

26. Rare finding • reported in less than 2% Australian Aboriginals • difficult to see on dark skin • Presence of rash diagnostic of ARF • Pale center and darker margins • Blanch under pressure • Circular snake like pattern • Occurs on trunk and extremities • Not itchy or painful Erythema marginatum

27. Rare, only seen in 2% cases • Highly specific of ARF • Strongly associated with carditis • Round firm and freely mobile • 0.5 to 2.0 cm in diameter • Appear 1 to 2 weeks after symptom onset • Occur in crops of up to 12 • over elbows knees, wrists, ankles, achilles tendons, occiput, and posterior spinal processes Subcutaneous nodules

28. Minormanifestations • High risk groups • Low Risk groups • Polyarthralgia or aseptic monoarthritis • Monoarthralgia • Fever • Fever • ESR≥30 mm/h or CRP ≥30 mg/L ESR≥30 mm/h or CRP ≥30 mg/L • ECG changes • ECG changes

29. Fever • Temperature greater than 38C • In the absence of fever documentation • reliable history if anti-inflammatory therapy given already given

30. ESR & CRP • Repeat serology 10 to 14 days if not confirmatory • To satisfy minor criteria: • serum CRP ≥30mg/L • ESR ≥30mm/hr • Elevated WBC insensitive marker for ARF

31. ECG • If ARF suspected always ECG • Check P-R interval • Normal 0.16 sec if 3 to 12 years old • If prolonged • repeat ECG in 1 to 2 months • If P-R interval returns to normal: • ARF more likely

32. Diagnosis: key investigations

33. Differential diagnosis

34. Diagnosis key points • ARF remains a difficult diagnosis • requires recommended tests to be performed • High index of suspicion for populations at greatest risk • Cardiology opinion recommended for suspected ARF • In high risk populations also consider ARF if: • child < 5 years of age presents with arthritis • Monoarthritis is a common presentation • Simple falls rarely cause joint effusions • Hospital admission recommended for initial presentations

35. Probable ARF

36. First requires diagnosis then secondary prophylaxis • Inpatient assessment recommended • Specialist review for ongoing management • Bed rest • NSAIDs • Initial then follow up echocardiography • Chest x-ray • If heart failure: ACE inhibitors, diuretics • Consider steroids for carditis ARF diagnosis and management

37. Principles of secondary prevention • Secondary prevention first requires the diagnosis of ARF/RHD • Long term antimicrobial prophylaxis prevents recurrent ARF • but significant challenges in service delivery • Success requires: • register-based program • effective recall system • functioning primary health care service

38. Take home messages • Incidence in Aboriginal Australians and Torres Strait Islander people amongst the highest in the world • Predominantly affects children aged 5 to 15 • Largely affects disadvantaged populations • High index of suspicion in high risk populations • Diagnosis needs clinical criteria and investigation results • Diagnosis often requires hospital admission

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42. Diagnosis of ARF in children