Kawasaki disease is a disease of young children that causes a high temperature (fever), a rash, and other typical symptoms (listed below). Most children recover fully but serious complications develop in some cases. Early treatment can prevent complications.
What is Kawasaki disease and who gets it?
Kawasaki disease is an uncommon condition that mainly affects children aged under 5 years. It most commonly affects children aged 18-24 months. It causes various symptoms throughout the body (listed below). Kawasaki disease was first described in the 1960s by a Japanese doctor named Tomisaku Kawasaki. Since then cases have been reported in many countries.
What are the symptoms of Kawasaki disease?
A high temperature (fever) which lasts more than five days is usual. In addition, at least four of the following normally develop:
- Redness of the eyes (conjunctivitis).
- Changes in the mouth such as a red throat or tongue, or dry and cracked lips.
- A blotchy red rash. This normally fades within a week.
- Changes in the hands or feet such as mild swelling or redness. The skin often peels on some of the fingers or toes after about 2-3 weeks.
- One or more lymph glands in the neck become swollen.
Also, affected children are commonly very irritable. The diagnosis of Kawasaki disease is based on the above typical group of symptoms. There is no test to confirm the disease. Tests may be done to rule out other diseases that cause similar symptoms, such as measles, scarlet fever, etc.
Other symptoms may develop in addition to the above. These are less common and include:
- Being sick (vomiting).
- Tummy (abdominal) pains.
- Being off food.
- Joint swelling and pains.
- Skin going yellow (jaundice).
What causes Kawasaki disease?
The cause is not known. The symptoms seem rather like many common infections. However, it is not catching (contagious). It is rare for contacts of affected children also to become affected at the same time. This means that the cause is unlikely to be just an infection with a germ such as a bacterium or virus. It may be an unusual or severe reaction to a common virus that usually causes little or no harm to most people. But, no virus or other germ has been proved to cause this disease.
There seems to be some genetic tendency that increases the risk of developing the condition. For example, parents of children with Kawasaki disease are more likely to have had the condition themselves during childhood. Also, brothers and sisters of children with Kawasaki disease have an increased risk of developing the condition. So, a theory is that affected children may have a genetic make-up that makes them 'overreact' to a common viral infection that does not cause problems in most children.
How does the illness progress?
Typically, fever develops first and then the other symptoms (listed above) develop after a few days. Often, the symptoms that develop after the high temperature (fever) has started develop one after each other rather than all coming on at the same time. This is why the diagnosis of Kawasaki disease may take several days to make after the initial fever starts. In total, symptoms tend to last up to 10 days before easing and going, although complications sometimes develop (see below).
During the illness, some children become very unwell, but others have a mild illness. This may be similar to many common infections that are caused by a virus. However, any child suspected of Kawasaki disease is normally admitted to hospital, even if they appear to have a mild illness. A heart scan is normally done to look for complications (see below). If complications do occur, they usually develop a week or so after the symptoms listed above.
What are the possible complications of Kawasaki disease?
Without treatment, about 1 in 5 children who have Kawasaki disease develop inflammation of the blood vessels to the heart (coronary arteries). This can cause a swelling of a section of an artery, which is called an aneurysm.
A coronary artery aneurysm usually causes no symptoms. Over time it often goes away and the artery returns to normal. However, the wall of an aneurysm is weakened and abnormal. Serious problems may develop in some children with an aneurysm. The most serious is that a clot (thrombosis) may develop in the aneurysm. The muscle of the heart is supplied with oxygen, carried in the blood of these arteries. If a clot develops, the muscle does not get enough oxygen. The heart muscle can then be damaged, causing a heart attack. Currently less than 1 in 100 children with Kawasaki disease die of heart problems.
An aneurysm can be detected by a heart scan (an echocardiogram). If an aneurysm does occur, it starts to develop a week or more after the fever and other acute symptoms begin. Treatment within 10 days of symptoms starting often prevents an aneurysm developing. This is why it is important to diagnose and treat Kawasaki disease in the early stages of the illness.
The aneurysm of a coronary artery is caused by inflammation of the artery (a 'vasculitis'). This can occur in other arteries in various parts of the body - but is very rare. Various other rare problems have also been reported in some children with Kawasaki disease.
What is the treatment for Kawasaki disease?
- Aspirin. This is one of the few times aspirin is given to children. Aspirin helps to reduce the inflammation in the coronary arteries. It also helps to prevent clots in the blood.
- Immunoglobulin. This is an antibody mixture obtained from human blood. It is given by a slow injection over a few hours (infusion) into the bloodstream. It is not clear how it works. It may modify the response of the immune system in some way to prevent inflammation in the arteries.
Treatment with immunoglobulin given within 10 days of the onset of Kawasaki disease reduces the chance of heart complications. Without treatment, about 25 in 100 affected children develop an aneurysm. With treatment, this is reduced to fewer than 5 in 100.
Other treatments aim to make the child comfortable, such as giving lots to drink and nursing them through the acute feverish illness.
Research continues to search for better treatments. Some treatments which have been tried or are being studied for Kawasaki disease are:
Further research is needed to confirm the role of these medicines in the treatment of Kawasaki disease.
If aneurysms have developed, a specialist heart doctor for children (a paediatric cardiologist) will be involved. In this situation, treatment is needed to prevent heart attacks or other heart problems developing. The child will have regular heart scans to keep an eye on what is happening. He or she may need another medicine to stop a blood clot. Very occasionally an operation is needed on the heart.
What is the outlook (prognosis)?
Without treatment, most children make a full recovery but complications develop in some cases. With early treatment, complications are much less likely to develop.
Repeat heart scans are usually advised over several weeks. If the heart scan is normal at 6-8 weeks after symptoms began then long-term heart problems are thought to be unlikely.
Long-term follow-up (such as an annual check-up) may be advised if an aneurysm was detected, as the long-term effect on the heart is not yet fully known.
Further reading and references
Eleftheriou D, Levin M, Shingadia D, et al; Management of Kawasaki disease. Arch Dis Child. 2014 Jan99(1):74-83. doi: 10.1136/archdischild-2012-302841. Epub 2013 Oct 25.
Feverish illness in children - Assessment and initial management in children younger than 5 years; NICE Guideline (Updated August 2017)
Jamieson N, Singh-Grewal D; Kawasaki Disease: A Clinician's Update. Int J Pediatr. 20132013:645391. doi: 10.1155/2013/645391. Epub 2013 Oct 27.
Golshevsky D, Cheung M, Burgner D; Kawasaki disease--the importance of prompt recognition and early referral. Aust Fam Physician. 2013 Jul42(7):473-6.
I am P anca positive/mpo. i have had alot of issues and cannot be diagnosed. i am lost at what to do or where to turn. i was at Cleveland clinic and they just dont know. any thoughts appreciated.babs373
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