Drug Reduces Artery Damage in Kawasaki Disease

A drug that treats rheumatoid arthritis also shows potential for preventing artery damage caused by Kawasaki disease, the most common acquired cardiac disorder in U.S. children, according to a new study led by Moshe Arditi, MD, professor of pediatrics and biomedical sciences and director of the Infectious and Immunological Diseases Research Center at Cedars-Sinai.

The study was published online March 3 in the journal Arteriosclerosis, Thrombosis and Vascular Biology.

The cause of Kawasaki disease, which is diagnosed in about 4,000 U.S. children each year, is unknown. It is typically preceded by a fever and produces inflammation and injury of blood vessels. Life-threatening complications may result. Among them is an abnormal enlargement of coronary artery walls, or aneurysms, that can lead to thrombosis (blood clots), stenosis (artery narrowing) or rupture and may produce a heart attack. These aneurysms also may occur in systemic arteries, such as the abdominal aorta — part of the heart's large arterial trunk that supplies blood to the abdominal area — as well as arteries within the abdomen.

In their study, Arditi and his colleagues used a well-established mouse model of Kawasaki disease. They found that by administering anakinra, a drug approved by the U.S. Food and Drug Administration to treat rheumatoid arthritis, they could reduce not only coronary artery inflammation and injury but also abdominal aortic aneurysms in these mice. Anakinra targets a protein in the immune system, known as IL-1b, to block inflammation.

"Our results indicated that anakinra and related drugs may offer a promising novel therapeutic agent for treating Kawasaki disease, especially for patients who do not respond to current treatments," Arditi said. Researchers from several institutions besides Cedars-Sinai contributed to the study: the University of Tokyo in Japan; Cornell University in New York; UCLA; University of California, San Diego; and Rady Children's Hospital in San Diego.

The study was part of Arditi's ongoing efforts to develop new treatments for Kawasaki disease. He recently was awarded a five-year, $2.1 million grant by the National Institute of Allergy and Infectious Diseases to investigate the immunobiology and molecular mechanisms of the cardiovascular complications associated with Kawasaki disease.