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Kawasaki disease

Key sources
The following summarized guidelines for the evaluation and management of Kawasaki disease are prepared by our editorial team based on guidelines from the Italian Society of Pediatrics (ISP 2021), the Single Hub and Access Point for Pediatric Rheumatology in Europe (SHARE initiative 2019), and the American Heart Association (AHA 2017).
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Guidelines

1.Screening and diagnosis

Diagnostic criteria: as per ISP 2021 guidelines, diagnose typical KD when fever lasting > 5 days is associated with ≥ 4 of the following clinical criteria:
bilateral non-exudative conjunctivitis
changes of lips and oral mucosa
changes of the extremities and perineal region
polymorphous exanthema
cervical lymphadenopathy).
E
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2.Classification and risk stratification

Risk stratification: consider using echocardiographic coronary artery luminal dimensions converted to BSA-adjusted Z scores for risk stratification of coronary artery abnormalities.
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3.Diagnostic investigations

Electrocardiography: obtain ECG at baseline in all patients with suspected KD, as soon as the diagnosis is suspected.
B

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  • Echocardiography

  • Advanced imaging

  • Laboratory tests

4.Diagnostic procedures

Cerebrospinal fluid analysis: consider performing CSF analysis to rule out infectious meningitis.
C

5.Medical management

General principles: as per SHARE initiative 2019 guidelines, initiate treatment as soon as the diagnosis of complete or incomplete KD is made.
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  • IVIG

  • Aspirin

  • Corticosteroids

  • Monoclonal antibodies

  • Other immunosuppressive agents

  • Statins

  • Beta-blockers

6.Therapeutic procedures

Plasma exchange: as per SHARE initiative 2019 guidelines, do not perform plasma exchange in patients with KD, except on an individual basis after consultation with a specialist unit.
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  • Indications for revascularization

  • PCI

7.Surgical interventions

Coronary artery bypass graft: prefer CABG to PCI in patients with left main coronary artery disease, multi-vessel coronary artery disease with reduced LV function, multi-vessel coronary artery disease with lesions not amenable to PCI, and multi-vessel coronary artery disease in diabetic patients.
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  • Heart transplantation

8.Specific circumstances

Patients with coronary artery thrombosis: as per ISP 2021 guidelines, administer rtPA as first-line thrombolytic therapy in pediatric patients with KD complicated by coronary artery thrombosis.
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9.Patient education

General counseling
Consider providing general counseling regarding healthy lifestyle and activity promotion at every visit in all patients with or without coronary artery involvement.
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Educate patients and families that having had KD is part of the patient's permanent medical history.
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  • Physical activity counseling

  • Pregnancy and contraception counseling

10.Preventative measures

Thromboprophylaxis: as per ISP 2021 guidelines, initiate dual anti-platelet prophylaxis with low-dose aspirin (at a single dose of 3-5 mg/kg/day) and clopidogrel (at a single dose of 0.2 mg/kg/day in pediatric patients aged < 24 months and up to 1 mg/kg/day in pediatric patients aged ≥ 24 months) in patients with medium-sized coronary artery aneurysms or with multiple and complex aneurysms.
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  • Immunizations

11.Follow-up and surveillance

Hospital discharge and follow-up: consider discharging patients without coronary artery involvement from cardiology care at 4-6 weeks after disease onset. Consider obtaining ongoing follow-up to 12 months.
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  • Serial clinical assessment

  • Laboratory monitoring

  • Serial electrocardiography

  • Serial imaging

  • Surveillance angiography