Kawasaki Disease

This page is provided by Thomas J. A. Lehman MD

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Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with Kawasaki disease and related forms of vasculitis and rheumatic disese. He practices in New York City. Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

Kawasaki Disease is an acute vasculitis which typically affects young children. Most often the patients are young boys or girls in the first three years of life. After the age of 10 years Kawasaki Disease is extremely rare and the diagnosis should be regarded with suspicion.

The disease usually begins with a fever unresponsive to tylenol or aspirin which continues for at least 7 - 10 days . Many but not all children develop a swollen area on the neck which looks like an infection. Doctors usually recognize the disease when they see an unresponsive fever accompanied by a rash, dry cracked lips or other changes of the mouth or tongue, inflamed eyes (conjunctivitis), and arthritis or heart changes without other explanation.

The most important part of Kawasaki disease is its tendency to cause inflammation of the coronary arteries which supply blood to the heart. Changes can be found on echocardiogram in about 15% of patients. A few of these patients will have aneurysms (balloon like dilatations) which can be very serious. If one of the aneurysms becomes blocked or bursts the blood flow to the heart is interrupted and the child can have a 'heart attack.' Fortunately this is very rare and deaths due to Kawasaki disease are less than 5/1000.

Treatment for Kawasaki disease is intravenous gammaglobulin. Large doses of intravenous gammaglobulin are usually very effective in stopping the fever of Kawasaki disease and seem to limit or prevent aneurysm formation as well. It is very rare for a child not to improve with gammaglobulin. Although some children require two treatments, if there is no improvement the diagnosis should be reconsidered. Other diseases can be confused with Kawasaki disease which may require different therapy. Some doctors are reluctant to give gammaglobulin if the symptoms have been present for more than 10 days. However, gammaglobulin is still effective after the first 10 days, but the likelihood of preventing aneurysms is lower than if it is given during the first ten days. Recent studies have shown that corticosteroids are effective for many of the children who fail to respond appropriately to gammaglobulin.

Once the acute inflammation is brought under control the chronic symptoms of Kawasaki disease are usually controlled with nonsteroidal anti-inflammatory drugs like naproxen or ibuprofen. Many cardiologists and some rheumatologists still use aspirin, but the other nonsteroidal drugs are considered safer, easier to give and equally effective.

The etiology of Kawasaki disease is unknown. There have been many theories, but there is none which is generally accepted. It is not contagious to other children, although there are a few cases of brothers and sisters developing the disease. In rare cases a few children have had two episodes. The disease often occurs in epidemics which occur every few years. This may be an epidemic year (I have had a lot more inquiries about this disease during the winter of 2002).

Usually children recover completely with proper therapy. There is some concern that children with damaged coronary arteries will have problems later in life, but this seems to be very rare. If your child has Kawasaki disease he will need to be followed by his primary doctor, a cardiologists, and perhaps a rheumatologist or other specialist (depending on the symptoms). The vast majority of children with Kawasaki disease recover and resume normal lives. Your doctor should be able to tell you if there is any reason to be unusually concerned. If your child has not recovered appropriately after an episode of Kawasaki disease it is important to be sure that the diagnosis is right and that another disease requiring different therapy has not been missed. A pediatric rheumatologist is the specialist best able to do this for you.

It is impossible to put all of the information I think you should know on a web page. I’ve spent the last year completing a book for the parents of children with rheumatic diseases. Included are chapters on Kawasaki Disease and other forms of vasculitis which may be mistaken for Kawasaki Disease, understanding the laboratory tests, and getting the best care for your child. If you want more information, check it out now.

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Reviewers’ Comments

“Dr. Tom Lehman’s experience and compassion are evident on every page of this book, and they help guide the reader—child, parent, and healthcare professional alike – through the world of childhood arthritis. This book is an absolute gem written with a single goal in mind: improve the lives of kids with arthritis.” -- Jack Klippel, M.D. President and CEO of the Arthritis Foundation

“Dr. Lehman has given parents and families of children with arthritis the first book that speaks to the parent and child as equals. His book explains the illnesses, the medications, the lab tests, and the disease course in simple, understandable lay language and givens them valuable insight into how a pediatric rheumatologist thinks. Bravo!”-- Charles Spencer, M.D., Professor of Clinical Pediatrics, University of Chicago, La Rabida

It’s not just growing pains.
A guide to childhood muscle, bone, and joint pain,
rheumatic diseases and the latest treatments

Click here to see the table of contents

It has always been a frustration trying to answer the many questions I have received from people over the web. I can’t take the time and give them the detail I would like to. I have to take care of my patients. This book is a distillation of my experience answering questions for parents and health professionals over 25 years of practice. If you want to know about the diseases, the tests, the medications, or how to be sure you are getting the best care– If you are the family member of a child with joint pains, this book will give you the answers. If you are a general physician, a pediatrician, or a nurse who cares for children with these diseases it will answer many of the questions families ask you, and you can recommend it to them. It will also answer many of your questions about what shots to give, what precautions to take, and the other questions families, pediatricians, and other health care providers have asked me over the years.

Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with rheumatic diseases including SLE, JRA, dermatomyositis, scleroderma, Kawasaki disease and related conditions. He practices in New York City. Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

Dr. Lehman is the author of many textbook chapters and articles on the care of children and young adults with SLE. He practices in New York City. Click here for more information about Dr. Lehman or the Hospital for Special Surgery.

The Arthritis Foundation works with children with KD and so does the American Heart Association.

Click for BOOKS dealing with childhood rheumatic diseases.

This site provided by Thomas J. A. Lehman MD
Chief, Division of Pediatric Rheumatology
The Hospital for Special Surgery
535 E 70 St, New York, NY 10021
212-606-1151, fax 212-606-1938, e-mail goldscout@aol.com

There are many thoughts regarding the etiology of Kawasaki disease, but no proof of its etiology. For some personal thoughts see the next page.

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