Kawasaki Disease and MIS

By: Sabrina and Anumita

Kawasaki Disease (KD), known also as Kawasaki Syndrome or Mucocutaneous Lymph Node Syndrome (MLNS) is a rare but severe febrile illness which typically manifests in children younger than 5 years of age. KD is the leading source of acquired heart disease in children in the United States. It induces inflammation in the heart region and is known to target the coronary arteries, which are responsible for providing blood to the heart. Kawasaki Disease happens in 3 distinct phases; the phases are dependent on when the disease is diagnosed and treated, causing a prolongment or shortage in its duration.

HISTORY

Kawasaki Disease was first discovered by Dr. Tomisaku Kawasaki, to which the disease was named after. He was the first person to classify the disease as a separate syndrome in 1967, the time of which he was a practicing pediatrician in the Japanese Red Cross Medical Centre. Since then, he has made a name for himself as a renowned pioneer in the discovery, research, and treatment of KD. Dr. Tomisaku Kawasaki spent 40 years dedicated to his craft and was a regular speaker at conferences concerning the disease. The first case found outside of Japan was in Hawaii during 1976. From then on, the disease spread around the world and was regularly found in clusters among children. A fellow doctor described Dr. Kawasaki as “part Sherlock Holmes and part Charles Dickens”, with his “vivid descriptions” and “detailing of every conceivable aspect of the disease.” Sadly, Dr. Tomisaku Kawasaki passed away on June 5th, 2020, forever leaving behind a prominent legacy in the medical field.

WHO DOES IT AFFECT?

Research and observations gathered throughout many outbreaks resurfaced around the world in the late 20th century. In the present day, many pediatricians and scientists have recognized the disproportionate allocation of cases in patients based on several factors. First off, evidence points to boys being 1.5 times more likely to be affected when compared to girls. Also, children under the age of 5 and those who hail from Japan or Asian ethnic groups are more susceptible to the disease. Although the average age of those affected with Kawasaki Disease is 2, KD can occur in any person, no matter their background or age. Because of the severity of the illness and the high risk of complications, most children are immediately hospitalized once they are diagnosed.

In the United States, the number of KD cases and hospitalizations are as follows:

5,447 people with KD are hospitalized each year
- 75% of KD cases are found in children under the age of 5
4,200 children are diagnosed with KD annually
19 out of 100,000 children have KD

CAUSES

Kawasaki Disease has no discernible cause as of yet, but over the years, there have been many theories suggested by the scientific community. As many outbreaks occur during the transition period between winter and spring, many theorize that KD has something to do with environmental factors like allergens and particles from fungi, that come along with the change in seasons. Others say it is a combination of genetics and a hyperactive immune response to bacterial or viral infections.

SYMPTOMS/COMPLICATIONS

Kawasaki Disease has a multitude of symptoms and some cases only experience a few of them. However, a long-lasting fever that is 102.2 F (39 C) or above is a definitive sign. Fevers associated with KD typically last up to 5 days. KD inflames the blood vessels throughout the whole body and targets the coronary arteries. This can cause devastating damage to one’s heart, proving fatal if proper treatment is not administered quickly. Kawasaki Disease happens over the course of 3 stages.

1st stage (lasts up to 2 weeks):

Fever
Rash in the torso area
Swollen hands/feet
Redness in the eyes (resembles pink eye)
Swelling of lymph glands
Inflammation of the mouth region

2nd stage (lasts up to 2 weeks):

Peeling of skin on hands/feet
Diarrhea
Vomiting
Abdominal pain
Joint pain (temporary arthritis)

3rd stage (usually ends six weeks after stage one began):

The symptoms begin to recede unless complications arise

Kawasaki Disease can be a very deadly illness for some. A few people who have KD and receive treatment still experience complications. The recurrence of this disease is at a minimal 3%.

Complications that can happen:

Coronary artery dilations - the weakening of the artery walls
Aneurysms - a bulge in the wall of an artery
- 25% chance of coronary aneurysm per KD case
- Aneurysms can lead to blood clots which in turn increase the risk of a heart attack or internal bleeding
Arrhythmias - irregular heartbeat
Inflammation of heart muscle
- 50% chance of developing it

TREATMENTS

When Kawasaki Disease is found and diagnosed early, the majority of the patients recover fully One dose of immunoglobulin is given, which greatly reduces the risk of coronary artery aneurysms. In occasional cases where immunoglobulin isn’t effective, steroids are given in its place. Steroids are an alternative drug to immunoglobulin as they are the next best thing that can reduce the risk of heart complications. Lastly, aspirin is taken via the mouth by the patient throughout the recovery process to treat the inflammation. However, the aspirin shouldn’t be used for long in pediatric patients as it has ties to a rare but deadly disease called Reye’s syndrome, which induces swelling of the brain and liver. With Reye’s syndrome compounded on top of KD, this can lead to higher mortality rates and possible negative health effects in the future.

KAWASAKI-LIKE DISEASE DURING COVID-19

During this coronavirus pandemic which has currently been running rampant throughout the world, there have been reports of a multisystem inflammatory syndrome in children (MIS - C) with symptoms that look similar to KD. This rare syndrome has appeared in little spurts around the globe with it mainly affecting older children. There is a possible connection to the pandemic as children with MIS - C have either tested positive for coronavirus or its antibodies. Currently, MIS - C has three phenotypes. The first one being Gastrointestinal with abdominal pain and diarrhea being the prominent symptoms. The second type is a Kawasaki-like one. As its name suggests, it shares the same symptoms as the Kawasaki Disease. The last type is Neurological. Patients who suffer from this category of MIS - C, show symptoms of headaches, seizures, and confusion. In recent studies, researchers have found that Kawasaki Disease has no apparent connection to the coronavirus pandemic but more research is needed. This new inflammatory disease that shares some similarities with KD can be separated by a few key differences. Firstly, MIS - C affects the functioning of the heart while KD predominantly affects the coronary arteries. Additionally, MIS - C patients exhibit more symptoms relating to the gastrointestinal part of the body and show increased inflammation throughout the body when compared to KD. As for treatments, it is very similar to Kawasaki disease with the administration of immunoglobulin and steroids. Nevertheless, since this syndrome is a newly found disease, things are still changing every day, and scientists are learning and adapting to them as they happen.

IN CONCLUSION

Kawasaki Disease is a relatively new disease that was first documented in 1967. Since then scientists have determined the common symptoms of the disease, but are yet to find the cause. KD can be very deadly if it is left untreated as it attacks the heart and causes inflammation throughout the whole body. However, children who are given the standard treatment of immunoglobulin and aspirin recover fully within eight weeks of contracting the illness. Currently, scientists are still conducting studies and research to better understand KD.

Works Cited

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Kawasaki Disease and MIS - C