Kawasaki Disease

Basics

Kawasaki disease (KD) is a medium-sized vessel arteritis of early childhood with a predilection for the coronary arteries, which can result in dilatation, aneurysms, thrombosis, and stenosis.
No diagnostic test exists, and incomplete presentations are common. Prompt recognition and treatment can reduce risk of coronary artery involvement from 25% to 5%.

U.S. hospitalization data (2003, 2006, 2009, and 2012) demonstrate an annual incidence of 18 to 20.8/100,000 children aged <5 years and 6.11 to 6.64/100,000 children aged 0 to 19 years.
KD is more common in boys than girls. In 2012, the U.S. hospitalization rate for boys was 7.24/100,000 versus 5.11/100,000 in girls.
More common in Asian/Pacific Islanders, with highest rates in Japan
- Ethnic predisposition persists in different geographic locations, with highest incidence in the United States in Asian/Pacific Islander children (10.3/100,000).
- Rates are higher in black and Hispanic children compared to Caucasian children, but most KD patients in the United States are Caucasian.

5,033 reported cases of KD in the United States in 2012

Patients of Asian/Pacific Islander descents are at higher risk of KD.
Patients who are younger and/or refractory to standard therapy are at higher risk of coronary artery disease.

Siblings have 10- to 30-fold higher risk of KD.
Genome-wide linkage studies suggest polymorphisms in multiple genetic loci involved in immune response may confer susceptibility to KD.

Generalized arteritis with early neutrophilic infiltrate, with later transition to lymphocyte infiltration, and lastly to luminal myofibroblastic proliferation
Can very rarely result in destruction of the endothelium through to the adventitia resulting in aneurysms and the rare possibility of rupture

Etiology is unknown.
Infectious cause suggested by the following:
- Abrupt onset and resolution of symptoms, usually without recurrence
- Clusters and epidemics
- Age of affected patients
- Seasonal predominance
- Oligoclonal IgA plasma cells noted in KD tissues, which bind to cytoplasmic inclusion bodies found in affected tissues
Data suggest that KD is caused by a previously unrecognized ubiquitous RNA virus that causes disease in a genetically susceptible population.
- No supporting evidence for multiple proposed etiologic agents: toxic shock toxin, rug shampoo, retrovirus, bocavirus, coronavirus, mercury, Epstein-Barr virus (EBV)/cytomegalovirus (CMV)

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