diagnosis of arf in children. speakers november 2012 alan ruben fracp, fafphm paediatrician and...
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Diagnosis of ARF in children
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Speakers
November 2012
Alan Ruben FRACP, FAFPHMPaediatrician 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, 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.
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Learning objectives
November 2012
• 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
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Take home messages
November 2012
• 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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Abbreviations
November 2012
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
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6November 2012
More information – Guidelines
www.rhdaustralia.org.au
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More information – Quick reference
November 2012
www.rhdaustralia.org.au
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More information – other modules
November 2012
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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
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• Amongst the highest rates in the world
• ARF commonest in remote and disadvantaged areas
• Some Australian medical staff unfamiliar with ARF
Australian setting
November 2012
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11November 2012
Environment
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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
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GAS pharyngitis
Arthritis
Carditis
Chorea
Fever
Exaggerated immune response
Acute rheumatic fever – ARF
November 2012
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ARF recurs - often many times
Valve damage is cumulative and silent
Rheumatic heart disease (RHD)
Cardiac failure, early death
*
November 2012
ARF progression
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Jones criteria
November 2012
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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
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Major manifestations
November 2012
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Major manifestations
High risk groups
Polyarthritis or aseptic mono-arthritis or polyarthralgia
Carditis (including subclinical evidence of rheumatic valvulitis on echocardiogram)
Chorea
Erythema marginatum
Subcutaneous nodules
Low Risk groups
Polyarthritis
Carditis
Erythema marginatum
Subcutaneous nodules
Chorea
November 2012
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• 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
Arthritis
November 2012
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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
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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
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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
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Carditis: investigations
• Early echocardiography essential
- repeated at 2 to 6 weeks
• Chest x-ray
• Electrocardiogram
November 2012
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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
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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
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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
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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
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Minor manifestations
High risk groups
Monoarthralgia
Fever
ESR≥30 mm/h or CRP ≥30 mg/L
ECG changes
Low Risk groups
Fever
ESR≥30 mm/h or CRP ≥30 mg/L
ECG changes
Polyarthralgia or aseptic monoarthritis
November 2012
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Fever
• Temperature greater than 38C
• In the absence of fever documentation
- reliable history if anti-inflammatory therapy given
already given
November 2012
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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
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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
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Diagnosis: key investigations
November 2012
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Differential diagnosis
November 2012
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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
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Probable ARF
November 2012
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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
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Principles of secondary prevention
November 2012
• 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
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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
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November 2012
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November 2012
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November 2012
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Diagnosis of ARF in children