What Is Kawasaki Disease?

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As news has spread of an inflammatory condition affecting children, possibly linked to COVID-19, many parents have become understandably worried. The condition, named “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19” by The New York State Department of Health, has affected at least 73 children as of May 8, 2020, and may be linked to other cases across the country as well as in the United Kingdom and Europe.

One defining characteristic of the condition is that its symptoms resemble many of the symptoms of Kawasaki disease. “This syndrome has features which overlap with Kawasaki Disease and Toxic Shock Syndrome,” explains a May 2020 memo from The New York State Department of Health. “Inflammatory markers may be elevated, and fever and abdominal symptoms may be prominent.”

As such, many concerned parents out there are wanting to know more about Kawasaki disease, including symptoms, causes, when to worry – and of course, the link between Kawasaki disease and COVID-19. We have answers.

What Is Kawasaki Disease?

Definition

Kawasaki Disease (also known as Kawasaki syndrome) is a condition predominantly affecting children under the age of 5, but older children and adults may be affected as well. It primarily involves inflammation of the blood vessels, and includes symptoms such as red, swollen eyes, lips and mouth; swelling and reddening of the hands and feet; and swollen lymph nodes. In more severe cases, cardiac symptoms can develop.

Although the cause is still unknown, Kawasaki is characterized by abnormal immune system activation. It is believed that an infectious trigger in those with a genetic predisposition plays a role. Kawasaki disease is not contagious. With treatment, the majority of people with Kawasaki disease are able to recover.

Characteristics, Symptoms, and Complications

According to the National Institutes of Health (NIH), the “classic” characteristics of Kawasaki disease include swelling of the mouth, eyes, and lips, as well as a rash on the hands and feet. Many sufferers will also have swollen lymph nodes. 

But there are many other symptoms that have been linked to Kawasaki disease.

Most Common Symptoms

Eighty percent to 99% of people with Kawasaki disease will have these symptoms, according to NIH:

  • Lip inflammation
  • Pink eye (conjunctivitis)
  • Swollen Lymph Nodes (lymphadenopathy)
  • Reddening of the skin (erythema)
  • Protein in the urine
  • Sore throat
  • Rash on skin
  • Inflammation of blood vessels (vasculitis)

Other Common Symptoms

Thirty percent to 79% of people will have these symptoms, according to NIH:

  • Stomach/abdominal pain
  • Abnormalities to the heart valve muscle
  • Changes in fingernail color
  • Joint inflammation (arthritis)
  • Diarrhea
  • Fluid retention (edema)
  • Fever over 102.2
  • Fatigue
  • Tongue inflammation
  • Increased white blood count
  • Pericarditis (swelling of the heart membranes)

Less Common Symptoms

The following symptoms are a sampling of symptoms that do occur, but with less frequency—affecting 5% to 29% of people with Kawasaki disease, according to the NIH. Although less common, these symptoms are among the most serious, and often require medical attention and/or hospitalization.

  • Abnormal heart rate
  • Pain in the joints
  • Inflammation of the gallbladder
  • Congestive heart failure
  • Cranial nerve paralysis
  • Inflammation of the liver
  • Jaundice
  • Meningitis
  • Inflammation of the heart muscle
  • Nausea and vomiting

Other notable symptoms that often affect children, according to the Kawasaki Disease Foundation, include a fever lasting at least five days; dry, cracked lips; and peeling of the skin on the hands, feet, fingers, and toes.

Causes of Kawasaki Disease

At this time, the exact cause of Kawasaki disease is not known. The disease is characterized by inflammation in the blood vessels, but what triggers this inflammation is unclear.

Experts believe that various viruses may trigger it, but the National Institutes of Health (NIH) explains that as of now, no specific virus or infection has been identified. Additionally, genetics may play a role, though experts are not certain of what that role may be. The disease may also be an immunological response, says NIH.

Risk Factors for Kawasaki Disease

The following factors may make you more susceptible to Kawasaki disease:

  • Genetics: The ITPKC gene may play a role in Kawasaki disease, according to the National Institutes of Health (NIH). “Researchers suggest that the ITPKC gene variation may interfere with the body's ability to reduce T cell activity, leading to inflammation that damages blood vessels and results in the signs and symptoms of Kawasaki disease,” NIH explains.
  • Age: Children under the age of five are more susceptible to Kawasaki disease, although older children and teens also get diagnosed with it. Though rare, adults can have Kawasaki disease as well.
  • Ethnicity: The disease more typically affects children of Asian or Pacific Island descent. According to NIH, for example, about 1 in 10,000 children under 5 get Kawasaki disease in the United States and Western countries, whereas the disease is 10-20 times more likely to strike a child of East Asian descent.
  • Other risk factors: Sex may play a role who gets Kawasaki disease, as boys are slightly more likely to be affected. In addition, family trends may play a role here. For example, according to NIH, children whose parents have had Kawasaki disease are twice as likely to get it; if their siblings had it, they are ten times as likely to get it. 

Diagnosis

There is no one specific diagnosis tested for Kawasaki disease. If you suspect that your child has it, you should contact your doctor right away for an assessment.

Here’s what may happen when you visit your doctor:

  • Your doctor will rule out other diseases with similar symptoms to Kawasaki disease, such as toxic shock syndrome, scarlet fever, measles, and juvenile rheumatoid arthritis.
  • Your doctor may order blood tests to look at your child’s white blood cell count, iron levels, and inflammation.
  • Your doctor may perform an electrocardiogram or echocardiogram. to look for abnormalities in the way your child’s heart is functioning.

Treatment

The good news is that with treatment, most cases of Kawasaki disease are treatable. Although a small number of children do die from Kawasaki disease, most survive with prompt and proper treatments.

Usually, your doctor will want to begin treatment as soon as a diagnosis is made, and hospitalization is usually required. Here are some of the treatments that will be employed:

  • Gamma globulin treatments are the most common treatments for Kawasaki disease. Gamma globulin proteins are administered intravenously to your child, usually in high doses. According to NIH, improvement is usually seen within 24 hours of treatment.
  • Aspirin treatments are also given in most cases, as it decreases inflammation, reduces swelling, and brings down fever. Lose dose aspirin administration may be given for weeks after initial treatment.

Note: As a general rule, aspirin should not be given to children, because of the risk of Reye's syndrome. Aspirin should only happen under the strict guidance of a doctor.

In rare cases, your child may develop blood vessel or heart problems as a result of Kawasaki disease. In these cases, your child will be referred to a pediatric cardiologist, who may recommend specific treatments.

The Link Between Kawasaki Disease and COVID-19

At this time, the information we have about Kawasaki-like disease linked to COVID-19 is very new and constantly evolving. As of this writing, doctors in New York as well as around the country have seen numerous concerning cases of children exhibiting signs of Kawasaki disease, toxic shock syndrome, as well as other concerning symptoms.

Pediatric Multi-System Inflammatory Syndrome

The hypothesis so far is that these mysterious inflammatory symptoms, which present very similarly to Kawasaki disease, are now known as “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19” and may be linked to COVID-19 infection.

The reason doctors suspect a link to COVID-19 is that most patients presenting with this new syndrome have either tested positive for COVID-19 or tested positive for antibodies of the virus (meaning that they may have previously been infected with it).

“The majority of patients who have presented with this syndrome have tested positive for SARSCOV-2 or corresponding antibodies,” explains a May 6th, 2020 memo from the New York State Department of Health. “Some tested positive on diagnostic, molecular testing for SARS-COV-2, others were positive on serological testing for corresponding antibodies.”

The New York State Department of Health is urging pediatricians who come across children with these symptoms to promptly refer families to specialists, as this syndrome requires immediate expert care and hospitalization. “Early recognition by pediatricians and prompt referral to an in-patient specialist, including to critical care is essential,” explains the memo.

In addition, it’s important to understand that as “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19” is not the same as Kawasaki syndrome, although it has many similar features.

As Dr. Steven Kernie, of Columbia University and NewYork-Presbyterian Morgan Stanley Children’s Hospital, explains to The New York Times, this new syndrome doesn’t affect the heart in the same way as Kawasaki syndrome.

Whereas Kawasaki disease is known to cause coronary aneurysms, “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19” primarily causes coronary artery inflammation, as well as inflammation of blood vessels, explains Dr. Kernie. This new condition also causes symptoms of shock, which is not generally seen with Kawasaki disease.

A Word From Verywell

These are very scary times to be living through, especially as a parent. This new inflammatory syndrome affecting children has certainly got parents worried and feeling on edge.

Again, please note that “Pediatric Multi-System Inflammatory Syndrome Potentially Associated with COVID-19” is brand new and doctors are still trying to understand its seriousness as well as its origins. Either way, if your child exhibits any concerning symptoms, including symptoms similar to Kawasaki syndrome, contact your pediatrician as soon as possible.  

Keep in mind, too, that children usually do experience mild symptoms of COVID-19. That hasn’t changed with the emergence of this concerning new condition. As always, if you have any questions or concerns about your child, this new inflammatory disease. Kawasaki disease, or COVID-19 in general, your best best is to contact your doctor. They are there to answer your questions, and to help keep your child healthy.

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Article Sources
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  1. New York State Department of Health. Health advisory: Pediatric multi-system inflammatory syndrome associated with coronavirus disease (COVID-19) in children. Updated May 6, 2020.

  2. Centers for Disease Control and Prevention. About Kawasaki Disease. Updated May 19, 2020.

  3. Genetic and Rare Diseases Information Center. Kawasaki disease. Updated March 9, 2017.

  4. Kawasaki Disease Foundation. The Symptoms of Kawasaki Disease. 2020.

  5. U.S. National Library of Medicine. Kawasaki disease. Updated May 12, 2020.

  6. KidsHealth from Nemours. Kawasaki Disease. Updated April 2020.

  7. New York State Department of Health. Childhood Inflammatory Disease Related to COVID-19. Updated May 24, 2020.