Kawasaki’s disease, also known as Kawasaki’s syndrome, is an autoimmune disease primarily affecting children under the age of five. A pediatrician in Japan, Tomisaku Kawasaki, first described the disease in 1967. It was not until 1976 that the first cases were reported outside of Japan in Hawaii. In 2006 the number of hospitalizations in the US for Kawasaki’s was 5523.
Kawasaki’s disease affects organ systems especially those affecting skin, mucous membranes, lymph nodes, and especially the blood vessels. This disease presents with a multiple of symptoms. Symptoms include, but are not limited to:
I. A high fever for more than five days that does not respond to antibiotics,
II. Reddening of the eyes (conjunctivitis) without puss,
III. A rash on the body, predominantly on the palms of the hands and the soles of the feet, the rash hardens and peels,
IV. Cracked and inflamed lips and mucous membranes in the mouth with an inflamed “strawberry tongue”,
V. Irritability,
VI. Ulcerative gum disease (gingivitis),
VII. Joint pain,
- Cough and runny nose,
IX. Swollen lymph nodes in the neck (cervical lymphadenopathy).
Kawasaki’s disease causes inflammation in the walls of the small and medium-sized arteries (vasculitis) throughout the body, including the coronary arteries that supply blood to the heart muscle. Vasculitis can cause weakening of the blood vessels and lead to areas of vessel widening (aneurysms). Coronary aneurysms occur in as many as 25% of Kawasaki’s cases. Special attention is paid to the hearts of children with Kawasaki’s. Testing includes electrocardiogram (EKG) and ultrasound (echocardiogram) to see any damage to the coronary arteries.
Arteries of the lungs, neck, and abdomen may also become inflamed. From these, patients may experience breathing problems, headaches, and belly pain. Less common findings include inflammation of the lining of the heart (pericarditis), joints (arthritis), or covering of the brain and spinal cord (meningitis). Blood tests are run throughout treatment to measure the degree of inflammation.
Treatment of Kawasaki’s disease consists of gammaglobulin administered through the vein together with fluids. This treatment can lessen the odds of aneurysms developing in the coronary arteries. Also used are high doses of aspirin (salicylic acid) to reduce inflammation and thin blood mildly to prevent clotting. The patient may be required to maintain an aspirin regimen and receive regular echocardiograms if there is coronary artery damage. Ibuprofen or naproxen may be used to treat joint pain. Patients not responding to aspirin or gammaglobulin have been reported to respond to Plasma exchange (plasmapheresis). Plasmapheresis is a procedure where plasma is removed from the blood and replaced with protein-containing fluids.
Kawasaki’s disease usually clears itself out after four to eight weeks and with treatment a full recovery is typical. In a minuscule amount of patients, death can occur from blood clots forming in abnormal areas of aneurysms of the coronary arteries. Some children develop larger aneurysms; they have a dimmer prognosis than the typical case.
Since we do not know what causes Kawasaki’s Disease we do not know of any preventative measures. Being aware of the disease and recognizing symptoms for early diagnosis and treatment is our best defense for now.
