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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Kawasaki disease, also known as lymph node syndrome, mucocutaneous node disease, infantile polyarteritis, and Kawasaki syndrome, is a poorly understood self-limited vasculitis that affects many organs, including the skin, mucous membranes, lymph nodes, blood vessel walls, and the heart. There is no evidence that Kawasaki disease is contagious. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan. Kawasaki disease is predominantly a disease of young children, with 80% of patients younger than 5 years of age. Additional risk factors in the United States include Asian race and male sex. Kawasaki disease can cause vasculitic changes (inflammation of blood vessels) in the coronary arteries and subsequent coronary artery aneurysms( reported in about 25% of cases). Common symptoms of Kawasaki disease include high-grade fever, red eyes, bright red and cracked lips, red mucous membranes in the mouth, strawberry tongue, white coating on the tongue or prominent red bumps (papillae) on the back of the tongue, red palms of the hands and soles of the feet, swollen hands and feet, and rash. Intravenous immunoglobulin(IVIG) and aspirin are indicated in Kawasaki disease.

Historical Perspective

Kawasaki disease was first discovered in 1961 by Tomisaku Kawasaki, a Japanese pediatrician. He published his first case report in 1967 in the Japanese language. He founded the Kawasaki Disease Research Center in 1990.

Pathophysiology

The pathophysiology of Kawasaki disease is not well understood. Most current theories are immunological origin. It is primarily an inflammation of the medium-sized blood vessels affecting multiple organ systems. This can lead to various complications such as pericarditis, pneumonitis, myocarditis, aseptic meningitis, coronary artery aneurysms, etc. It leads to chronic inflammation of blood vessels which can evolve into stenosis as well as aneurysms. On microscopic analysis, ciliated bronchial epithelial cells frequently show intracytoplasmic inclusion-bodies.

Genetics

While genetic predisposition has been observed in Kawasaki disease, the pattern of inheritance is unclear. The Children, as well as the siblings of Kawasaki disease patients, are at an increased risk of developing the disease.