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Kawasaki Disease Awareness Day
Monday 26th January 2026

In recognition of International Kawasaki Disease Day (26th January), OPTIMAL CRE is pleased to share the Top 10 Kawasaki Disease Questions, developed in response to the many questions submitted by families and health professionals at the Kawasaki Disease Information Day for Families and Health Practitioners held last year. The Information Day, hosted by OPTIMAL CRE in partnership with the Murdoch Children's Research Institute, brought together clinicians, researchers, and community members to support greater awareness and understanding of Kawasaki Disease.

While time did not allow for all questions to be addressed on the day, we have since worked closely with our expert presenters to compile clear, evidence-based responses to the most commonly raised questions. We sincerely thank our medical advisors and health practitioners for the time and care they have taken in preparing these answers, and the Kawasaki Disease Foundation for their continued collaboration in supporting KD awareness and education.

The Top 10 KD Questions and Answers are now available (see below or on the Kawasaki Disease Foundation website) and form part of our ongoing commitment to improving access to trusted information for families and clinicians.

Kawasaki Disease Information Day Top 10 Questions

Question 1.

How is KD diagnosed?

Kawasaki disease (KD) is a clinical diagnosis; there is no specific diagnostic test. The diagnosis is made by a child having a prolonged fever of 4 or more days together with 4 or 5 well-recognised clinical diagnostic features. These features are (i) skin rash; (ii) conjunctival inflammation (redness of the eyes); (iii) changes to the mouth and throat (red lips, strawberry tongue, red throat); (iv) redness and swelling of the hands and feet; peeling of the skin around the nails later in the illness; and (v) enlarged lymph nodes (glands) in the neck.

These features come on sequentially over a number of days and often are not all present at the same time. In some children, especially infants, the diagnosis can be made even if there are less than 4 clinical features present. In these cases, blood and other tests may provide additional information, particularly if they show high levels of inflammation and exclude other causes for fever. About one third of children with KD will also have another infection, such as a viral infection at the same time.

The diagnosis of KD can be difficult and may therefore take some time while other causes are excluded.

If KD is diagnosed, then an ultrasound of the heart and coronary arteries (echocardiogram or echo) is performed. However, an echo is not usually used as a diagnostic test as it is normal in three quarters of children with untreated KD.

Question 2.

What causes KD? Is there a genetic basis or link to having vaccines?

The cause of KD has been one of the enduring mysteries of paediatrics; we still are not sure what causes it.

However, it is generally agreed that KD results from more than type of common infection triggering abnormal inflammatory responses by the child’s immune system. This only happens in a minority of children and there is good evidence that some children are genetically more likely to develop KD if they come across the infectious triggers for KD.

There has been lots of research to identify a specific infection as the cause of KD (including COVID-19), but this has been negative. Also, efforts to identify non-infectious triggers, such as vaccines or chemicals, have not shown any link with KD.

KD is not contagious to other children. The risk of KD in siblings of children with KD, although modestly increased, is still low. It is important to remember that KD is a relatively uncommon condition; there is one new case diagnosed each day in Australia.

Question 3.

Is KD painful?
What are the symptoms?

Children with KD are frequently described as being “irritable” which means that they are upset and difficult to settle. This is probably a combination of the high fever and some of the symptoms which are likely to be painful. The most common symptoms such as conjunctivitis (red eyes), mucositis (red lips), lymphadenopathy (swollen lymph glands) or red swollen hands are all caused by inflammation which is an immune process known to be associated with pain. It is difficult to tell if the skin rash is painful for children with KD.

Less commonly, children have inflammation in other areas such as their joints causing arthritis (joint pain), the external lining of their brains (sometimes called aseptic meningitis as it is not caused by infection but more an inflammatory reaction) which can cause headache. Other complications of KD, including gall bladder swelling and blockage (hydrops of the gall bladder), may also cause pain in the initial stages.

The good news is that the inflammatory changes are very responsive to standard treatments for KD and usually will settle quickly providing symptomatic relief for children with KD.

The coronary artery changes are not thought to cause pain unless the blood supply to the heart is reduced and the blood supply to the heart muscle is reduced but this is a very rare complication.

Question 4.

What is IVIg, where does it come from and why is it used in KD?

Immunoglobulins are antibodies, and part of the immune system. In health, their main role is to help fight infections.

Immunoglobulins are collected from plasma – the liquid part of the blood – donated by blood donors.

Immunoglobulins can be used for treatment of a wide range of conditions. In some people with low immunoglobulin levels, replacing immunoglobulins to normal levels can reduce the risk of infection. These treatments may be administered either intravenously (through a vein) or subcutaneously (under the skin).

High-dose intravenous immunoglobulins (IVIg) can also be effective treatment for many other conditions, including some with inflammatory, immune, or infectious causes, or where the underlying cause is not known.

Kawasaki Disease is one of these conditions and IVIg is a mainstay of current therapy. Under the National Blood Authority Criteria, treatment of KD is approved for access to government-funded immunoglobulin therapy, at no direct charge to the patient.

Currently there is no synthetic (artificial) alternative to plasma-derived IVIg, and thousands of plasma donations need to be processed to make one batch. Clinical demand is growing, and Australia now imports over half the immunoglobulins we use. IVIg products are also very costly, in part reflecting the many steps required for their production and to make sure they are as safe as possible. These include careful blood donor selection, testing of donated plasma, treatment of the plasma and the final product during preparation to reduce infectious and other risks, and careful documentation of all steps in the process.

Ongoing supply of plasma is essential to make sure that there is enough IVIg for patients who need it. In Australia, you can check if you are eligible to donate plasma at: https://www.lifeblood.com.au/donors/blood-plasma-platelets/making-a-donation

More information on how immunoglobulins are managed in Australia is available here:

https://www.blood.gov.au/immunoglobulin-therapy

and a useful video is available here:

https://www.blood.gov.au/immunoglobulin-videos-clinicians-and-patients

Question 5.

What are the risks/rate of re-occurrence and needing another round of treatment?

After the first treatment with intravenous immunoglobulin (IVIg), about one-third of children may need additional treatment in the following days to weeks to fully settle that episode of KD. This may include a second dose of IVIg or other medicines to help reduce inflammation and support recovery.

Once that episode of KD has completely resolved, the chance of a child developing KD again is very low. Studies show that only a small number of children (less than 2%) ever experience a second episode.

Question 6.

If a child has recovered from KD and their heart is normal, are there any other health problems they might face later in life, like autoimmune conditions?

If a child was appropriately treated with timely IVIg, had no coronary artery involvement, and inflammation fully resolved, most children will not experience ongoing problems related to KD.

KD does not appear to significantly increase the lifetime risk for other autoimmune conditions (such as lupus, rheumatoid arthritis, inflammatory bowel disease or multiple sclerosis). Large follow-up studies have not shown a consistent or strong association, and the immune system changes have not been shown to result in clinically meaningful disease if no recurrent or chronic inflammation occurs.

Children who fully recover typically have normal immune function after resolution and do not have higher rates of infections, allergies or chronic inflammatory conditions. If ongoing inflammation or recurrent KD episodes occur, re-evaluation with a specialist including counselling may help you understand these issues specifically for your child and family.

For all children whose echocardiograms (an ultrasound scan that shows how the heart muscle and heart vessels are working) are normal during and after KD, long-term cardiovascular outcomes are considered similar to the general population.

As clinicians, we would recommend that children and families take a proactive approach to long-term cardiovascular health and wellbeing to improve lifelong cardiometabolic risk. This includes heart-healthy habits including regular exercise, balanced diet, avoiding smoking and vaping, screening and management for diabetes and high blood pressure, manage cholesterol levels, avoid alcohol consumption and maintaining a healthy weight.

If, however, there was evidence of during or after KD of coronary artery dilatation (widening), aneurysms (see question 7) or heart dysfunction, children are considered to be at an increased lifetime risk of heart complications and the advice of your local cardiologist should be followed.

Stay Updated on Immunoglobulin Research in Australia

optimalcre
Jul 22, 2024
2 min read

Are you interested in staying up to date with the latest advancements in immunoglobulin research in Australia? Look no further than Optimal CRE, a Center for Research Excellence that is at the forefront of immunoglobulin research in the country.

Optimal CRE is committed to bringing together researchers from all corners of Australia to collaborate and innovate in the field of immunoglobulin research. Their website serves as a hub for all things related to their work, providing a wealth of resources for those interested in learning more about this important area of study. One of the key features of the Optimal CRE website is its news section, which regularly updates visitors on the latest research findings, breakthroughs, and developments in the field of immunoglobulin research. By staying tuned to this section, visitors can stay informed and engaged with the latest advancements in the field. In addition to the news section, the website also features an events calendar that highlights upcoming conferences, seminars, and other events related to immunoglobulin research. This is a great way for researchers and stakeholders to stay informed about opportunities to network, collaborate, and learn from others in the field. For those looking to dive deeper into the world of immunoglobulin research, the website's resource library is a treasure trove of research publications and presentations from Optimal CRE-affiliated studies. Visitors can access a wealth of information that can help inform their own research and contribute to the advancement of the field. Overall, the Optimal CRE website is a valuable resource for anyone with an interest in immunoglobulin research in Australia. By staying updated on the latest research, events, and resources available through the website, visitors can be at the forefront of this important area of study.