Review of Kawasaki Disease

Newburger JW, Takahashi M, Burns JC.
Kawasaki Disease. J Am Coll Cardiol 2016;67:1738-1749.

The following are key points to remember about this review on Kawasaki disease (KD):

  1. Kawasaki disease (KD) is an acute, self-limited vasculitis occurring mostly in infants and young children. KD is characterized initially by high fever, mucocutaneous inflammation, and cervical lymphadenopathy.
  2. Approximately one in five children not treated with intravenous immunoglobulin (IVIG) in the acute phase of illness will develop coronary artery aneurysms (CAAs).
  3. The precise etiology of KD remains unclear. It is believed that KD is due to an immunologic reaction elicited in genetically susceptible hosts after exposure to environmental triggers.
  4. Recent data suggest that the trigger for KD is carried by large-scale tropospheric winds from provinces in northeastern China. This may service as a source region for the seasonal clustering and annual epidemic of KD cases in Japan, Hawaii, and southern California (Rodó X, Curcoll R, Robinson M, et al. Proc Natl Acad Sci USA 2014;111:7952-7).
  5. Echocardiography is the primary cardiac diagnostic modality during the acute phase of illness, and should be performed at baseline as well as at 1-2 weeks and 4-6 weeks after treatment.
  6. Aneurysm size is determined by the internal luminal dimension and is categorized as small (≤4 mm), medium (>4 to ≤8 mm), and giant (>8 mm). Echocardiographic measurements may also be normalized on the basis of body size, with small aneurysms having a z-score of ≥2.5 to <5, medium aneurysms ≥5 to <10, and giant aneurysms either ≥10 or >8 mm in diameter.
  7. IVIG-resistant patients may be treated with a second infusion of IVIG, either alone, or in combination with steroids and infliximab. A retrospective study compared second IVIG infusion with infliximab for retreatment. The infliximab group had more rapid resolution of fever and inflammatory markers, shorter hospital stay, and lower costs of care, although there was no difference in coronary artery outcomes.
  8. Risk factors for development of CAA include male sex, age <6 months, age >8 years, and those with laboratory studies suggesting worse systemic inflammation. IVIG resistance also predicts higher risk of aneurysm formation.
  9. Myocardial infarction occurs most commonly in the first year after disease onset, with the highest risk period being 15-45 days after disease onset.
  10. Goals of long-term management are to prevent coronary thrombosis and treat myocardial ischemia and associated complications.
  11. For patients with normal or only transiently dilated coronary arteries (z-score <2.5), aspirin therapy may be stopped at 4-8 weeks once inflammatory markers and a reassuring follow-up echocardiogram have been obtained. A lipid profile should be performed 1 year after KD onset, and longer-term follow-up should emphasize modification of coronary artery disease risk factors.

Clinical Topics: Congenital Heart Disease and Pediatric Cardiology, Noninvasive Imaging, Stable Ischemic Heart Disease, Vascular Medicine, Atherosclerotic Disease (CAD/PAD), Congenital Heart Disease, CHD and Pediatrics and Imaging, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Echocardiography/Ultrasound, Chronic Angina

Keywords: Aspirin, Coronary Aneurysm, Coronary Artery Disease, Coronary Thrombosis, Echocardiography, Heart Defects, Congenital, Immunoglobulins, Intravenous, Infant, Inflammation, Mucocutaneous Lymph Node Syndrome, Myocardial Infarction, Myocardial Ischemia, Risk Factors

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