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Breakthrough Toward Finding Cause of Kawasaki Disease in Children

March 12, 2020

After more than 50 years of fruitless searching, scientists offer first insight into the cause of Kawasaki disease

Research from Ann & Robert H. Lurie Children’s Hospital of Chicago identified for the first time a segment of a new protein sequence that might be causing Kawasaki disease, the leading cause of acquired heart disease in children. Although the disease was first described in the 1960s, its cause has remained elusive – until now. Establishing the exact cause, which is believed to be viral, is critical to advancing the diagnosis, treatment and prevention of Kawasaki disease. Findings were published in the Journal of Infectious Diseases.

“We finally have the first indication of what the cause of Kawasaki disease might be,” says lead author Anne Rowley, MD, pediatric infectious diseases specialist at Lurie Children’s and Professor of Pediatrics (Infectious Diseases and Microbiology-Immunology) at Northwestern University Feinberg School of Medicine. “We discovered a small protein sequence that is targeted by the antibodies that kids with Kawasaki disease produce as part of their immune response. This is a highly promising breakthrough toward determining a causal agent of the disease.”

Kawasaki disease is relatively uncommon, affecting mostly children between 6 months and 5 years of age. Lurie Children’s sees 50-60 newly diagnosed Kawasaki disease patients a year.

Clinical signs include fever, rash, swelling of the hands and feet, irritation and redness of the whites of the eyes, swollen lymph glands in the neck, and irritation and inflammation of the mouth, lips, and throat. Children with Kawasaki disease have a 30 percent chance of developing heart disease, while infants are at higher risk with 50 percent chance of cardiac complications. The standard treatment, intravenous immunoglobulin and aspirin, substantially decreases the risk of heart disease in Kawasaki disease patients. Steroids may be added for highest risk patients.

“Once we establish the cause of Kawasaki disease, we can work on developing a vaccine to prevent these infections in children,” says Dr. Rowley. “Knowing the cause also will help us diagnose the disease and develop more precise treatment. At this stage, our team at Lurie Children’s is building on our findings and trying to map the entire protein sequence that may be causing Kawasaki disease. We do not yet know, however, if the disease is caused by single or multiple agents.”

Dr. Rowley stresses that her work would not have been possible without strong partnerships with families of children with Kawasaki disease.

“The families want answers and they’ve put their trust in this research,” she says. “We have the responsibility to keep searching, to deliver those answers and optimize diagnosis, treatment and prevention of Kawasaki disease.”

This work was supported in part by National Institute of Allergy and Infectious Diseases R21 grant (AI140029) to Dr. Rowley.

Research at Ann & Robert H. Lurie Children’s Hospital of Chicago is conducted through the Stanley Manne Children’s Research Institute. The Manne Research Institute is focused on improving child health, transforming pediatric medicine and ensuring healthier futures through the relentless pursuit of knowledge. Lurie Children’s is ranked as one of the nation’s top children’s hospitals by U.S. News & World Report. It is the pediatric training ground for Northwestern University Feinberg School of Medicine. Last year, the hospital served more than 220,000 children from 48 states and 49 countries.