Despite the prominent mucocutaneous clinical findings that define the illness, Kawasaki disease is best

regarded as a generalized vasculitis that involves small- to medium-sized arteries. Although the vascular
inflammation is most pronounced in the coronary vessels, vasculitis can also occur in veins, capillaries, small
arterioles, and larger arteries.
In the earliest stages of the disease, the endothelial cells and the vascular media become edematous, but the
internal elastic lamina remains intact. Then, approximately 7-9 days after the onset of fever, an influx of
neutrophils occurs, which is quickly followed by a proliferation of CD8 + (cytotoxic) lymphocytes and
immunoglobulin Aproducing plasma cells.
The inflammatory cells secrete various cytokines (ie, tumor necrosis factor, vascular endothelial growth factor,
monocyte chemotactic and activating factor), interleukins (ILs; ie, IL-1, IL-4, IL-6), and matrix
metalloproteinases (MMPs; ie, primarily MMP3 and MMP9) that target the endothelial cells and result in a
cascade of events that eventuates in fragmentation of the internal elastic lamina and vascular damage. [17]
In severely affected vessels, the media develops inflammation with necrosis of smooth muscle cells. The
internal and external elastic laminae can split, leading to aneurysms.
Over the next few weeks to months, the active inflammatory cells are replaced by fibroblasts and monocytes,
and fibrous connective tissue begins to form within the vessel wall. The intima proliferates and thickens. The
vessel wall eventually becomes narrowed or occluded owing to stenosis or a thrombus. [18, 19, 20, 21, 22]Cardiovascular
death may occur from a myocardial infarction secondary to thrombosis of a coronary aneurysm or from rupture
of a large coronary aneurysm.
Most of the pathology of the disease is induced by a medium vessel arterial vasculitis. Initially, neutrophils are
present in great numbers, but the infiltrate rapidly switches to mononuclear cells, T lymphocytes, and
immunoglobulin A (IgA)producing plasma cells. Inflammation involves all 3 layers of vessels. Eosinophils are
preferentially accumulated in microvessels.
The period during of the greatest vascular damage is when a concomitant progressive increase in the serum
platelet count occurs, and this is the point of the illness when the risk of death is most significant.
Endothelin-1 (ET-1), pentraxin 3 (PTX3), N-terminal pro-brain natriuretic peptide, and brain natriuretic peptide
(BNP) are made and secreted from vascular and/or myocardial tissue. They are also useful in the prediction of
coronary artery lesions formation and IVIG non-responsiveness and in KDs acute phase. Some biomarkers
can help evaluate atherosclerosis and chronic coronary arteritis and KDs convalescent phase. [23]

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