AHA Scientific Statement on Kawasaki Disease

McCrindle BW, Rowley AH, Newburger JW, et al.
Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation 2017;Mar 29:[Epub ahead of print].

The following are key points to remember from this Scientific Statement, which serves as an update to the 2004 American Heart Association guidelines for the diagnosis, treatment, and long-term management of Kawasaki disease (KD):

  1. A recently proposed model of KD vasculopathy involves three processes impacting muscular arteries. The first is a necrotizing arteritis, followed by subacute/chronic vasculitis. The final process is luminal myofibroblastic proliferation.
  2. The diagnosis of complete KD is based on the presence of ≥5 days of fever and ≥4 principal clinical features (extremity changes, rash, conjunctivitis, oral changes, and cervical lymphadenopathy).
  3. The current statement includes an algorithm for the evaluation of suspected incomplete KD. This algorithm begins with a child with fever ≥5 days and two or three compatible clinical criteria, or infants with fever ≥7 days without other explanation. A C-reactive protein ≥3.0 mg/dl and/or erythrocyte sedimentation rate (ESR) ≥40 mm/hr are supportive of KD. Patients with these laboratory findings and 1) a positive echocardiogram, or 2) ≥3 supportive laboratory findings (anemia for age, platelet count of ≥450,000 after the seventh day of fever, albumin ≤3.0 g/dl, elevated alanine aminotransferase (ALT), white blood cell count ≥15,000/mm3, or urine with ≥10 white blood cell/hpf) should be treated.
  4. Echocardiography should be performed when the diagnosis of KD is considered, and repeated at 1-2 and 4-6 weeks after treatment.
  5. Quantitative assessment of luminal dimensions should be performed and normalized as Z-scores adjusted for body surface area. This is in contrast to previous guidelines, which used absolute dimensions. Z-scores are classified as follows:
    • No involvement: Always <2
    • Dilation only: 2 to ≤2.5; or if initially <2, a decrease in Z-score during follow-up ≥1
    • Small aneurysm: ≥2.5 to <5
    • Medium aneurysm: ≥5 to <10, and absolute dimension <8 mm
    • Large or giant aneurysm: ≥10, or absolute dimension ≥8 mm
  6. Patients should be treated with intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion, usually given over 10-12 hours. Administration of moderate (30-50 mg/kg/day) to high (80-100 mg/kg/day) dose of aspirin should be continued until the patient is afebrile.
  7. Single-dose pulse methylprednisolone should not be administered with IVIG as primary therapy. Administration of a longer course of corticosteroids, together with IVIG and aspirin, may be considered for treatment of high-risk patients with acute KD when such high-risk patients can be identified prior to initiation of treatment.
  8. Approximately 10-20% of patients with KD have persistent or recurrent fever after primary therapy with IVIG plus aspirin. The statement details the role of additional therapeutic options, including a second dose of IVIG, high-dose pulse steroids, longer course of steroids, infliximab, cyclosporine, and immunomodulatory monoclonal antibody therapy.
  9. Risk classification of coronary artery abnormalities is based on the Z-score descriptions above. The five groups are subdivided based on the behavior of the aneurysms. For example, group 3 (small aneurysms) is subdivided in to 3.1 (current or persistent) and 3.2 (decreased to dilatation only or normal luminal dimension).

Keywords: Anemia, Aneurysm, Arteritis, Aspirin, Body Surface Area, C-Reactive Protein, Conjunctivitis, Coronary Vessels, Cyclosporine, Echocardiography, Heart Defects, Congenital, Immunoglobulins, Intravenous, Leukocytes, Methylprednisolone, Mucocutaneous Lymph Node Syndrome, Polyarteritis Nodosa, Vasculitis

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