Kawasaki Disease

What is Kawasaki disease?

From: MedlinePlus, National Library of Medicine

Kawasaki disease is a rare illness that usually affects small children. Other names for the disease are Kawasaki syndrome and mucocutaneous lymph node syndrome. It is a type of vasculitis, which is inflammation of the blood vessels. Kawasaki disease is serious, but most children can fully recover if they are treated right away.

What causes Kawasaki disease?

From: MedlinePlus, National Library of Medicine

Kawasaki disease happens when the immune system injures the blood vessels by mistake. Researchers do not fully know why this happens. But when it does, the blood vessels become inflamed and can narrow or close off.

Genetics may play a role in Kawasaki disease. There may also be environmental factors, such as infections. It does not seem to be contagious. This means that it cannot be passed from one child to another.

Who is more likely to develop Kawasaki disease?

From: MedlinePlus, National Library of Medicine

Kawasaki disease usually affects children under the age of 5. But older children and adults can sometimes get it. It is more common in boys than girls. It can affect children of any race, but those with Asian or Pacific Islander descent are more likely to get it.

What are the symptoms of Kawasaki disease?

From: MedlinePlus, National Library of Medicine

The symptoms of Kawasaki disease may include:

• High fever lasting at least five days
• A rash, often on the back, chest, and groin
• Swollen hands and feet
• Redness of the lips, lining of the mouth, tongue, palms of the hand, and soles of the feet
• Pink eye (conjunctivitis)
• Swollen lymph nodes in the neck

Contact your child's health care provider if your child has a fever for 4 days, especially if they have any other symptoms of Kawasaki disease.

What other problems can Kawasaki disease cause?

From: MedlinePlus, National Library of Medicine

Sometimes Kawasaki disease can affect the walls of the coronary arteries. These arteries bring blood and oxygen to your heart. This can lead to:

• An aneurysm (bulging and thinning of the walls of the arteries). This can raise the risk of blood clots in the arteries. If the blood clots are not treated, they could lead to a heart attack or internal bleeding.
• Inflammation in the heart.
• Heart valve problems.

Kawasaki disease can also affect other parts of the body, including the brain and nervous system, the immune system, and the digestive system.

How is Kawasaki disease diagnosed?

From: MedlinePlus, National Library of Medicine

There is no specific test for Kawasaki disease. To find out if your child has Kawasaki disease, your child's provider:

• Will do a physical exam, which includes looking at the signs and symptoms
• Will likely order blood and urine tests to rule out other diseases and check for signs of inflammation
• May order tests to check for damage to the heart, such as an echocardiogram and electrocardiogram (EKG)

What are the treatments for Kawasaki disease?

From: MedlinePlus, National Library of Medicine

Kawasaki disease is usually treated in the hospital with an intravenous (IV) dose of immunoglobulin (IVIG) antibodies. Antibodies are proteins that your immune system makes to fight infections.

Aspirin may also be part of the treatment. But do not give your child aspirin unless the health care provider tells you to. Aspirin can cause Reye syndrome in children. This is a rare, serious illness that can affect the brain and liver.

Treatment usually works. But if it is not working well enough, the provider may also give your child other medicines to fight the inflammation. If the disease affects your child's heart, they might need additional medicines, surgery, or other medical procedures.

Genetic causes described in the linked summary

From: MedlinePlus Genetics

The causes of Kawasaki disease are not well understood. The disorder is generally regarded as being the result of an abnormal immune system activation, but the triggers of this abnormal response are unknown. Because cases of the disorder tend to cluster geographically and by season, researchers have suggested that an infection may be involved. However, no infectious agent (such as a virus or bacteria) has been identified.

A variation in the ITPKC gene has been associated with an increased risk of Kawasaki disease. The ITPKC gene provides instructions for making an enzyme called inositol 1,4,5-trisphosphate 3-kinase C. This enzyme helps limit the activity of immune system cells called T cells. T cells identify foreign substances and defend the body against infection. Reducing the activity of T cells when appropriate prevents the overproduction of immune proteins called cytokines that lead to inflammation and which, in excess, cause tissue damage. Researchers suggest that the ITPKC gene variation may interfere with the body's ability to reduce T cell activity, leading to inflammation that damages blood vessels and results in the signs and symptoms of Kawasaki disease.

It appears likely that other factors, including changes in other genes, also influence the development of this complex disorder.

Inheritance described in the linked summary

From: MedlinePlus Genetics

A predisposition to Kawasaki disease appears to be passed through generations in families, but the inheritance pattern is unknown. Children of parents who have had Kawasaki disease have twice the risk of developing the disorder compared to the general population. Children with affected siblings have a tenfold higher risk.

Which doctor should you see?

The suggested department for discussing Kawasaki Disease is Rheumatology, with a rheumatologist as the relevant type of clinician. General physician / Family Medicine; paediatrician for children. Referral depends on symptoms.

Additional services that may be relevant, depending on the findings, include: Clinical Genetics.

This is an editorial referral starting point. The appropriate clinic depends on the person’s age, symptoms, previous diagnosis and local services. The first clinician can decide whether another specialty or a team is needed; a department label does not confirm the diagnosis.

How to prepare for an assessment

Bring a short timeline of the main symptoms: when they first appeared, whether they are constant or episodic, what seems to change them, and how they affect daily activities. Include previous reports, discharge summaries, current medicines and supplements, allergies, and any relevant family history. A dated record is more useful than trying to match every feature in an online article.

Ask the clinician what is already established and what remains uncertain. If a test is suggested, ask what question it answers, what its limitations are and how the result would change the next step. The information here is not an instruction to arrange every possible test. In children, bring growth, developmental and school information if it is relevant to the concern.

• Are the findings inflammatory, structural or due to another mechanism?
• Is there evidence that other organs need assessment?
• How will function and any treatment-related risks be monitored?

Treatment discussions and follow-up

Where the source describes treatments, these are an overview of possible care, not a prescription for an individual. Ask which option applies to the confirmed diagnosis, what benefit is expected, what adverse effects to watch for and how progress will be assessed. Availability, approvals and local practice can differ from the country described in the source.

Before leaving the appointment, clarify the next review date, who will communicate results, and whom to contact if the situation changes. Discuss difficulties with sleep, work, school, mobility, eating or emotional wellbeing when these are relevant. Practical support may require coordination between the treating clinician and other services.

The collected references do not establish a complete prevention or long-term outlook section for this entry. Missing information should not be interpreted as proof that prevention is impossible or that a particular outcome is inevitable. Ask what is known for the exact subtype, stage and personal circumstances, and which uncertainties remain.

When to seek emergency help

Severe breathing difficulty, collapse, new stroke-like symptoms, a seizure that is prolonged or repeated without recovery, uncontrolled major bleeding, or an immediate risk of self-harm require emergency help. In India, call 112 or reach the nearest emergency department. This is a general, non-exhaustive warning list; it is not a condition-specific triage tool.