Combo therapy may prevent blood vessel complications in children with Kawasaki disease

August 19, 2020

Research Highlights:DALLAS, August 19, 2020 -- Adding corticosteroids to standard intravenous (IV) immunoglobulin treatment for children with Kawasaki disease judged to be at higher risk of developing blood vessel complications made initial treatment more successful and prevented these complications, according to new research published today in the Journal of the American Heart Association, an open access journal of the American Heart Association.

"Early diagnosis and prompt treatment are important for children with Kawasaki disease to prevent the development of cardiac complications," said lead author Ryusuke Ae, M.D., Ph.D., an assistant professor in the department of public health at the Center for Community Medicine at Jichi Medical University in Shimotsuke, Japan. The study was conducted during 2018-2019 when Dr. Ae was a guest researcher at the U.S. Centers for Disease Control and Prevention (CDC) and is a collaboration between the CDC and Jichi Medical University.

Kawasaki disease, which occurs most often in children younger than 5 years old, causes inflammation of the blood vessels, particularly the coronary arteries that supply fresh blood to the heart muscle. In developed countries, Kawasaki disease is a leading cause of heart disease in children born without heart defects and the cause is unknown. The criteria for diagnosis is when a child has a fever for five days or longer (unless interrupted by treatment) along with multiple other defining symptoms that can include a rash over the abdomen, swollen and red hands and feet, bloodshot eyes, swollen lymph glands, and redness and swelling of the mouth, lips, throat and tongue. Kawasaki disease occurs in children of all races and ethnicities; however, it is more common among Asian children regardless of where they live.

"As the blood vessel wall becomes enlarged, the inside of the vessel may narrow. Blood clots may form, blocking the artery and potentially leading to a heart attack. Children who have such vessel wall complications may require long-term follow-up after the onset of Kawasaki disease," said Ae.

Standard treatment for Kawasaki disease includes IV immunoglobulin with aspirin. However, for an estimated 17% of Kawasaki disease patients, initial IV immunoglobulin treatment is not effective, increasing their risk of cardiac complications. In recent years, it has become more common to add corticosteroids to the initial treatment approach; however, researchers have reached different conclusions about which approach is best.

In this study, the largest of its kind to-date, researchers analyzed real-world data on children with Kawasaki disease in Japan to determine whether the more intensive combination approach could heal children faster and prevent cardiac complications among those considered more vulnerable to treatment failure and long-term complications. In this study, patients were deemed at higher risk if initial treatment was predicted to be ineffective based on standing scoring systems, if the child was less than one year old or had elevated blood test results.

Researchers identified 1,593 Kawasaki disease patients under the age of 18 who were first treated with standard IV immunoglobulin with aspirin. Outcomes were compared with another set of 1,593 Kawasaki disease patients who were likely to have been initially treated with corticosteroids in combination with the standard therapy. Patients were matched for age, sex and how quickly treatment was started after symptoms appeared, with care taken to account for other factors that could bias results.

Compared with standard IV immunoglobulin treatment, the researchers found:"It was surprising to see the dramatic results of our analysis. Clinicians should consider initial combination treatment with multiple-dose corticosteroids for high-risk Kawasaki disease patients," Ae said.

Limitations of the study included that researchers did not have precise information on the type, dose and duration of corticosteroid therapy because of the way initial data was recorded.

The same combination treatment of immunoglobulin and corticosteroids has been recently used to treat children with multisystem inflammatory syndrome (MIS-C), a new condition associated with COVID-19 infection that has some symptoms like Kawasaki disease. While MIS-C is seen in children with COVID-19, it is rare.
Co-authors are Joseph Y. Abrams, Ph.D.; Ryan A. Maddox, Ph.D.; Lawrence B. Schonberger, M.D.; Yosikazu Nakamura, M.D., M.P.H.; Masanari Kuwabara, M.D., Ph.D.; Nobuko Makino, M.D., Ph.D.; Yuri Matsubara, M.D.; Koki Kosami, M.D.; Teppei Sasahara, M.D., Ph.D.; and Ermias D. Belay, M.D. The authors have no disclosures.

The study was funded by the Japan Kawasaki Disease Research Center.

Additional Resources:

Available multimedia is on right column of release link -

After Aug. 19, view the manuscript online.

What is Kawasaki disease?
Immunotherapy, steroids had positive outcomes in children with COVID-related multi-system inflammatory syndrome

Follow AHA/ASA news on Twitter @HeartNews
Follow news from the Journal of the American Heart Association@JAHA_AHA

Statements and conclusions of study authors published in American Heart Association scientific journals are solely those of the study authors and do not necessarily reflect the Association's policy or position. The Association makes no representation or guarantee as to their accuracy or reliability. The Association receives funding primarily from individuals; foundations and corporations (including pharmaceutical, device manufacturers and other companies) also make donations and fund specific Association programs and events. The Association has strict policies to prevent these relationships from influencing the science content. Revenues from pharmaceutical and device corporations and health insurance providers are available at

About the American Heart Association

The American Heart Association is a relentless force for a world of longer, healthier lives. We are dedicated to ensuring equitable health in all communities. Through collaboration with numerous organizations, and powered by millions of volunteers, we fund innovative research, advocate for the public's health and share lifesaving resources. The Dallas-based organization has been a leading source of health information for nearly a century. Connect with us on, Facebook, Twitter or by calling 1-800-AHA-USA1.

American Heart Association

Related Heart Disease Articles from Brightsurf:

Cellular pathway of genetic heart disease similar to neurodegenerative disease
Research on a genetic heart disease has uncovered a new and unexpected mechanism for heart failure.

Mechanism linking gum disease to heart disease, other inflammatory conditions discovered
The link between periodontal (gum) disease and other inflammatory conditions such as heart disease and diabetes has long been established, but the mechanism behind that association has, until now, remained a mystery.

New 'atlas' of human heart cells first step toward precision treatments for heart disease
Scientists have for the first time documented all of the different cell types and genes expressed in the healthy human heart, in research published in the journal Nature.

With a heavy heart: How men and women develop heart disease differently
A new study by researchers from McGill University has uncovered that minerals causing aortic heart valve blockage in men and women are different, a discovery that could change how heart disease is diagnosed and treated.

Heart-healthy diets are naturally low in dietary cholesterol and can help to reduce the risk of heart disease and stroke
Eating a heart-healthy dietary pattern rich in vegetables, fruits, whole grains, low-fat dairy products, poultry, fish, legumes, vegetable oils and nuts, which is also limits salt, red and processed meats, refined-carbohydrates and added sugars, is relatively low in dietary cholesterol and supports healthy levels of artery-clogging LDL cholesterol.

Pacemakers can improve heart function in patients with chemotherapy-induced heart disease
Research has shown that treating chemotherapy-induced cardiomyopathy with commercially available cardiac resynchronization therapy (CRT) delivered through a surgically implanted defibrillator or pacemaker can significantly improve patient outcomes.

Arsenic in drinking water may change heart structure raising risk of heart disease
Drinking water that is contaminated with arsenic may lead to thickening of the heart's main pumping chamber in young adults, according to a new study by researchers at Columbia University Mailman School of Public Health.

New health calculator can help predict heart disease risk, estimate heart age
A new online health calculator can help people determine their risk of heart disease, as well as their heart age, accounting for sociodemographic factors such as ethnicity, sense of belonging and education, as well as health status and lifestyle behaviors.

Wide variation in rate of death between VA hospitals for patients with heart disease, heart failure
Death rates for veterans with ischemic heart disease and chronic heart failure varied widely across the Veterans Affairs (VA) health care system from 2010 to 2014, which could suggest differences in the quality of cardiovascular health care provided by VA medical centers.

Heart failure: The Alzheimer's disease of the heart?
Similar to how protein clumps build up in the brain in people with some neurodegenerative diseases such as Alzheimer's and Parkinson's diseases, protein clumps appear to accumulate in the diseased hearts of mice and people with heart failure, according to a team led by Johns Hopkins University researchers.

Read More: Heart Disease News and Heart Disease Current Events is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to