Trial Summary

Kawasaki Disease is a disease where arteries, particularly the coronary arteries in the heart, become inflamed, sometimes causing irreversible heart damage, heart attacks or even death. Kawasaki Disease is currently the most common cause of acquired heart disease in childhood, and an important preventable cause of heart disease in the young. These heart complications may occur within a few weeks of disease onset, or more typically, some years after recovery due to the narrowing of the coronary arteries causing a lack of blood supply to the heart.

To prevent this heart damage, Kawasaki Disease in children and young people must be recognised by clinicians early, and promptly treated with anti-inflammatory medicines.

KD-CAAP is a multi-centre, randomised trial of corticosteroids plus standard of care treatment versus standard of care treatment alone to prevent heart complications in Kawasaki Disease. The trial is sponsored by University College London and is managed by the Medical Research Council (MRC) Clinical Trials Unit (KD-CAAP Co-ordinating Centre).

This study will work out the best way to treat children and adolescents aged between 30 days and 15 years who have Kawasaki Disease - whether the addition of corticosteroids to standard treatment is more effective than standard treatment alone.

In summary, in this study we will answer all of the following questions to work out the best way to treat children and adolescents aged 30 days to 15 years who have Kawasaki disease:

1. Does the combination of corticosteroid and IVIG/aspirin reduce the rate of heart complications in children/adolescents with Kawasaki disease across Europe? (main question)

2. Does the combination of corticosteroid and IVIG/aspirin reduce the length of stay in hospital for children/adolescents with Kawasaki disease, and do their blood test results improve faster?

3. What side effects do children/adolescents get with corticosteroids or other therapies to treat Kawasaki disease?

4. Is the combination of corticosteroid therapy and IVIG/aspirin a cost effective treatment for the management of Kawasaki disease?

All children and adolescents in the study will receive the current recommended standard treatment of IVIG and aspirin. They will then be split into two groups, by chance (called “randomisation”).

• The first group will receive the standard of care treatment of IVIG and aspirin only.
• The second group will receive additional treatment with prednisolone (corticosteroids) by mouth (or intravenously, into a vein, if needed) immediately. They will take steroids for around 2-3 weeks, depending on how quickly they get better.

Everyone will have frequent assessments of their inflammatory status (temperature and inflammatory blood test markers) to work out whether they still need additional IVIG treatment two days after they start treatment. Five days later, they will all be re-evaluated again to work out whether they still need extra treatment if the inflammation has not settled completely.

Whichever group they started in, children and adolescents will get any extra treatment they then need.

We will follow up with the children and adolescents for 12 weeks through face-to-face visits to find out whether they have had any problems – they will mostly stay in hospital for the first 5-7 days, and there are just three visits after this first week. This duration of follow-up is standard for routine clinical care of Kawasaki disease. We will particularly focus on:

• Looking for any damage to their coronary arteries (or other heart damage) using heart ultrasound (echocardiography) scans;

• Whether they experienced any side effects from the medicines they received;

• Whether they needed to receive any additional treatments; how long they had to stay in hospital;

• Whether they have to be admitted to hospital again;

• How rapidly their blood tests normalised;

• How much all their care costs; and

• If our treatments overall improve their quality of life.