Kawasaki Disease Research

Lurie Children’s has been a leader in clinical research on Kawasaki disease dating back to the treatment trials in the 1980s and early 1990s. Our clinician scientists are continuing their research efforts in finding the cause of this illness, improving treatment and identifying factors that determine susceptibility. As an active leading center, treatment trials were performed and a standard treatment plan was established. The treatment trials below brought us to our conclusions.

The first treatment trial proved that four days of intravenous immunoglobulin (IVIG) plus aspirin was highly effective in preventing coronary artery complications and resulted in prompt improvement in the fever, rash and other features of the illness.

The second treatment trial compared a single large dose of IVIG and aspirin to 4 days of IVIG treatment with aspirin and showed that large single dose IVIG resulted in faster recovery from fever, rash and other features of the illness.

The next treatment trial compared second dose IVIG to a medicine called infliximab for treatment of children who do not respond to the initial IVIG therapy. This trial revealed that both therapies could result in clinical improvement, and since there are more safety data using IVIG in children, we generally give a second dose of IVIG when it is clinically indicated.

Current Research

The most exciting research on the cause of Kawasaki disease is in the laboratory of Anne Rowley, MD​, where her research uses highly sophisticated tools of molecular biology. Dr. Rowley and colleagues have shown that there is a strong IgA immune response in children with Kawasaki disease. Using molecular immunology and studies with the high-powered electron microscope, her team has shown that IgA antibodies in children with Kawasaki disease are binding to abnormal structures present in cells lining the breathing tubes of Kawasaki disease children. These structures appear to represent accumulations of viral protein. Dr. Rowley’s research, which is largely funded by the National Institutes of Health and by philanthropic support, holds great promise to unravel the mystery surrounding the cause of Kawasaki disease. 

Lurie Children’s is also an active participant in the International Kawasaki Disease Registry (IKDR), an international registry consisting of pediatric referral centers across 5 different continents. The IKDR is one of the largest international cohorts of patients with coronary artery aneurysms. Our clinician scientists have published a large number of articles and delivered several medical lectures related to the clinical aspects of Kawasaki disease at courses, hospitals and symposia so other physicians and specialists can benefit from our extensive research.

These studies included:

  • Infants under one year of age with Kawasaki disease
  • Examining the changes in blood lipids (fats) in the early stages of Kawasaki disease
  • Two nationwide surveys of U.S. hospitals regarding the yearly number of hospital admissions for children with Kawasaki disease
  • Analyses of Japanese and American treatment studies using various IVIG and aspirin doses
  • Analysis of the efficacy of IVIG and aspirin to prevent giant coronary aneurysms
  • A study of the small number of patients worldwide with Kawasaki disease who have needed heart transplants
  • Study of the children who develop the very rare and severe complications of gangrene during Kawasaki disease
  • A study of several unusual genetic markers for the disease
  • Descriptions of children with Kawasaki disease who have swollen neck glands and fever as their only major sign of infection
  • A study of older children (8 years or older) with Kawasaki disease
  • A study of the rare children who require bypass surgery for complications of Kawasaki disease
  • A study of patients with Kawasaki disease who fail two doses of standard therapy
  • A study of Kawasaki disease patients who develop signs of shock requiring ICU care
  • A study of various tissue enzymes that contribute to development of coronary artery aneurysms in Kawasaki disease patients
  • A study of which inflammation proteins are increased in the coronary arteries of children with Kawasaki disease
  • A study of the specific antibody response to Kawasaki disease, which indicates a common immune response among patients to a single causative pathogen

To view more publications from our team, visit the links below: