Transcript Document
Diagnosis of ARF in children Speakers Alan Ruben FRACP, FAFPHM Paediatrician and Public Health Physician, Apunipima Cape 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, FRACP Paediatric 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. November 2012 2 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 November 2012 3 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 November 2012 4 Abbreviations AR aortic regurgitation ARF acute rheumatic fever BPG benzathine penicillin G CRP C-reactive protein ESR erythrocyte sedimentation rate GAS group A beta-haemolytic streptococcus MR mitral regurgitation RHD rheumatic heart disease November 2012 5 More information – Guidelines www.rhdaustralia.org.au November 2012 6 More information – Quick reference www.rhdaustralia.org.au November 2012 7 More information – other modules November 2012 8 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 November 2012 9 Australian setting • Amongst the highest rates in the world • ARF commonest in remote and disadvantaged areas • Some Australian medical staff unfamiliar with ARF November 2012 10 Environment November 2012 11 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 November 2012 12 Acute rheumatic fever – ARF Chorea Carditis GAS pharyngitis Exaggerated immune response Arthritis Fever November 2012 13 ARF progression * Valve damage is cumulative and silent ARF recurs - often many times Rheumatic heart disease (RHD) Cardiac failure, early death November 2012 14 Jones criteria November 2012 15 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 DNAse B • No other probable diagnosis November 2012 16 Major manifestations November 2012 17 Major manifestations High risk groups Polyarthritis or aseptic monoarthritis or polyarthralgia Carditis (including subclinical evidence of Low Risk groups Polyarthritis Carditis rheumatic valvulitis on echocardiogram) Chorea Chorea Erythema marginatum Subcutaneous nodules Erythema marginatum Subcutaneous nodules November 2012 18 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 November 2012 19 Can a monarthritis be ARF? • In high risk populations: - aseptic monoarthritis can be a major manifestation - monoarthritis often associated with carditis - if joint aspirate sterile, prior to treatment for septic arthritis, investigate for ARF November 2012 20 Polyarthralgia • A major criteria ONLY in high risk populations: - Multiple painful joints - Can be migratory - Unlike arthritis lacks: o Effusions o Heat o Morning stiffness November 2012 21 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 November 2012 22 Carditis: investigations • Early echocardiography essential - repeated at 2 to 6 weeks • Chest x-ray • Electrocardiogram November 2012 23 Carditis: treatment • Often requires inpatient bed rest and 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 November 2012 24 Sydenham’s chorea • Rapid, uncoordinated jerking movements • Primarily the face, feet and hands • Female to male ratio of 2:1 • Occurs up to 6 months after acute infection • Mostly children, 5 to 13 years • “Milkmaids” sign • Tongue fasciculations • Emotional lability November 2012 25 Erythema marginatum • 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 November 2012 26 Subcutaneous nodules • 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 November 2012 27 Minor manifestations High risk groups Monoarthralgia Fever Low Risk groups Polyarthralgia or aseptic monoarthritis Fever ESR≥30 mm/h or CRP ≥30 mg/L ESR≥30 mm/h or CRP ≥30 mg/L ECG changes ECG changes November 2012 28 Fever • Temperature greater than 38C • In the absence of fever documentation - reliable history if anti-inflammatory therapy given already given November 2012 29 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 November 2012 30 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 November 2012 31 Diagnosis: key investigations November 2012 32 Differential diagnosis November 2012 33 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 November 2012 34 Probable ARF November 2012 35 ARF diagnosis and management • 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 November 2012 36 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 November 2012 37 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 November 2012 38 More? November 2012 39 More? Register for… • Downloadable PowerPoint presentations • Additional resources • Additional assessment items for training providers www.facebook.com/RHDEd for notification about new modules and updates November 2012 40 How’d you go? Test your knowledge with a brief self-assessment quiz November 2012 41 Diagnosis of ARF in children