Emerging Inflammatory Disease In Children: What We Know So Far

In recent weeks, pediatricians in the UK and several European countries have reported cases of illness in children resembling Kawasaki Disease (KD), a rare childhood condition which involves prolonged fever, rashes, and inflammation seen in the eyes and mouth. Other features include lymph gland enlargement and swelling of the hands and feet. Laboratory studies in these children show systemic inflammation. Damage to the coronary arteries is among the most serious complications. Many of the children recently stricken tested positive for the virus that causes COVID-19.

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In the early days of the SARS-CoV-2 pandemic, experts observed that if children became infected at all, the course of the infection was milder, hospitalizations less frequent and mortality rare. This remains true. However, as of May 9, Governor Cuomo’s office is reporting that 3 children in NY have died and 73 others have been sickened with “pediatric multi-system inflammatory syndrome” or PMIS – the Kawasaki-like condition associated with COVID-19.

First described in Japanese children in 1967, KD has remained enigmatic in many ways. There is no single test for it (doctors rely on a combination of features) and its causes remain largely unknown. The KD variant now emerging in children and teens is no less mysterious. Is this merely a “look-alike” or true KD in a different form? Understanding this and other aspects of the condition will be important for doctors and families.

There are important differences between this new COVID variant and “classic” KD. COVID related cases are not only happening much more often than we would expect for KD generally, they also affect individuals in the first and second decade of life, while classic KD mostly attacks children younger than school age. Also, children with the COVID-associated syndrome often have abdominal pain and other intestinal symptoms that are not part of KD. Finally, shock (a critical condition in which there is insufficient delivery of blood flow and oxygen to vital organs) seems to occur much more often in the new condition than in classic KD.

Cardiac complications are among the most serious problems associated both with KD and PMIS. It was observed many years ago that children with KD sometimes went on to develop weaknesses in the wall of the coronary arteries, known as aneurisms. These aneurisms then set the stage for later heart attack when one of these arteries becomes blocked, leading in turn to the death of heart muscle cells, and problems ranging from a weak heart (“heart failure”) to sudden death. Fortunately, administration of a product known as intravenous immunoglobulin (IVIG) can cause dramatic improvement in KD cases and prevents the formation of aneurisms; early reports suggest that IVIG also works in the COVID-related multi-system inflammatory syndrome.

Despite concern for this new manifestation of COVID-19, Dr. Jane Newburger, M.D., M.P.H., FAHA, American Heart Association Young Hearts Council member and director of the Kawasaki Program at Boston Children’s Hospital recently noted that “We want to reassure parents – this appears to be uncommon. While Kawasaki disease can damage the heart or blood vessels, the heart problems usually go away in five or six weeks, and most children fully recover.”

What else do parents need to know? Though PMIS is uncommon, if a child develops gastrointestinal complaints, fever, rash, red eyes (without discharge), mouth inflammation and/or swelling of the hands and feet, parents should seek help promptly. A parent’s judgment is important. “If a parent feels that their child is looking unusually ill, they should listen to their instincts,” says Dr. Thomas McDonagh, a Huntington area pediatrician and Assistant Professor of Pediatrics at the Donald and Barbara Zucker School of Medicine at Hofstra/Northwell. Severe listlessness and irritability are often seen in KD.

Many parents are concerned about their children getting COVID-19 by going to a doctor visit or emergency room. However, Telehealth visits with a primary pediatrician are generally available and can help families and doctors decide whether further evaluation is warranted. “When in doubt, ask your trusted provider,” notes McDonagh.

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