Project Summary  

This research project is in progress. PCORI will post the research findings on this page within 90 days after the results are final.

What is the research about?

Kawasaki disease is a rare childhood health problem that inflames blood vessels. It can affect any artery or vein in the body. Symptoms include high fever; rash; dry lips; and swollen tongue, hands, and feet. The illness can sometimes cause a dangerous bulge, called an aneurysm, in the coronary artery.

No one knows what causes Kawasaki disease. Most children recover fully after treatment with intravenous immunoglobin, or IVIG. But 10 to 20 percent of children are resistant to IVIG treatment: The treatment doesn’t work well for them, and the fever comes back. These children have a higher risk of aneurysm. Doctors don’t know what treatment works best for them.

In this study, the research team is comparing two treatments for children whose Kawasaki disease comes back. One treatment is another dose of IVIG. The other is a medicine called infliximab.

Who can this research help?

Doctors and parents of children who have Kawasaki disease and are IVIG resistant can use results from this study when planning treatment.

What is the research team doing?

The research team is working with 30 hospitals across the United States to recruit 250 children who have Kawasaki disease and are IVIG resistant. The team is assigning IVIG-resistant patients by chance to receive either a second dose of IVIG or infliximab. Both are single treatments given at the hospital.

To see if patients’ fever goes away within 24 hours of starting treatment and stays normal for a week, the research team is following up with parents. The team is also tracking patients for

• Signs of infection
• Signs of aneurysm
• Total days with a fever after treatment
• Total days in the hospital
• Complications from treatment

Parents of children with Kawasaki disease and adults previously diagnosed with Kawasaki disease are working with the research team to plan and conduct the study. 

Research methods at a glance

Design Elements	Description
Design	Randomized controlled trial
Population	Children ages 4 weeks to 17 years who have a return of fever following initial treatment for Kawasaki disease
Interventions/ Comparators	IVIG infliximab
Outcomes	Primary: cessation of fever within 24 hours of treatment initiation, no recurrence within 7 days Secondary: cessation of fever within 24 hours of treatment completion, change in white blood cell count, absolute neutrophil count, and high-sensitivity C-reactive protein, change in Zworst score, total number of fever days; duration of hospitalization, treatment reactions and complications
Timeframe	7-day follow-up for primary outcomes

Journal Articles

Project Details

Principal Investigator

Jane Burns, MD

Other Principal Investigator

Katherine K. Kim, PhD, MPH, MBA

Project Status

In progress; Recruiting

Project Title

A Stakeholder-Driven Comparative Effectiveness Study of Treatments to Prevent Coronary Artery Damage in Patients with Resistant Kawasaki Disease

Board Approval Date

December 2016

Project End Date

February 2022

Organization

University of California San Diego

Year Awarded

2016

State

California

Project Type

Research Project

Health Conditions  

Cardiovascular Diseases

Rare Diseases

Intervention Strategies

Drug Interventions

Populations

Children -- 18 and under
Individuals with Rare Disease

Funding Announcement

Assessment of Prevention, Diagnosis, and Treatment Options

Project Budget

$2,326,223

Study Registration Information

HSRP20181533

NCT03065244

Partners

Patient/Caregiver Partners

• Greg Chin, KD Foundation

Other Stakeholder Partners

• No information provided by awardee.