Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 in children

As the COVID-19 pandemic underway in almost every country of the world, it has now been found that a novel paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection in children matches some symptoms of Kawasaki disease that predominantly affects children under the age of 5.


On 30th April 2020 this article was published mentioning that there has been a notable rise in children with “multisystem inflammatory state requiring intensive care” over the last 3 weeks in London and other UK regions. The cases had features of toxic shock syndrome and atypical Kawasaki disease. Symptoms displayed by children included a high temperature, abdominal pain as well as cardiac inflammation. This has been observed in children who tested SARS-CoV-2 negative and in those who tested SARS-CoV-2 positive.

As mentioned in that article, on 25th April 2020 Paediatric Intensive Care Society has issued a high priority alert over a possible SARS-CoV-2 related inflammatory syndrome in children to all GPs across the country. The advice to GPs read 'the prevalent presenting symptoms are abdominal pain and diarrhoea and rapid progress to a shock-like picture'. Cases seem to be rare at present and “Our understanding of pathophysiology is currently incomplete. Whether this is COVID-associated or something else is not clear at the moment.”

It is now classified as PMIS-TS(paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection) or MIS-C(multisystem inflammatory syndrome in children) and is characterised by a systemic inflammation with fever and organ dysfunction, which is thought to be linked to COVID-19. PMIS-TS or MIS-C matches some or all of the diagnostic criteria for Kawasaki disease about which we will talk in detail below.

RCPCH(Royal College of Paediatrics and Child Health) has issued guidance on PMIS-TS diagnosis and management.

So far around 100 cases of PMIS-TC have been reported in the UK, over 135 cases in France and over 100 cases in New York with a total of 5 fatalities. Within 6 weeks of SARS-CoV-2 arrival in Italy, doctors in Bergamo have reported a 30-fold increase in cases of Kawasaki-like disease.


What is Kawasaki Disease?

Kawasaki disease is characterised by a fever and it can affect children of any age; however, it mainly affects children under the age of 5. The disease was first identified by Tomisaku Kawasaki in Tokyo, Japan in January 1961.Today Kawasaki disease is recognized worldwide.


Kawasaki disease is 1.5 times more common in boys than in girls. If the condition is untreated, the complications of Kawasaki diseases are fatal in 2-3% of cases. Kawasaki disease is not contagious.


Signs and symptoms of Kawasaki disease

The symptoms of Kawasaki disease usually include:

  • High temperature 38C or more that lasts for 5 days or more and does not respond to ibuprofen or paracetamol

  • A skin rash

  • Red, dry and cracked lips

  • The whites of the eyes red and swollen

  • Red tongue and throat

  • Swollen lymph glands/nodes

During the first week of the onset of symptoms heart monitoring in the hospital will usually confirm tachycardia(rapid heart rate), myocarditis(heart muscle inflammation),aneurysms(coronary artery swelling) and pericardial effusion(a fluid collection in the heart). A blood test and a urine sample as well will be collected in order to confirm the diagnosis of Kawasaki disease.


After a week other symptoms can develop such as:

  • Diarrhea

  • Vomiting

  • Abdominal pain

  • Headache

  • Fatigue

  • Peeling skin

  • Yellow skin and yellow whites of the eyes


The symptoms will start gradually disappearing 4-6 weeks after the initial onset of symptoms.

Causes of Kawasaki disease

The cause of Kawasaki disease is not fully understood and it can be a combination of factors that leads to Kawasaki disease. Research suggests that it could be a combination of genes that provokes Kawasaki disease or a possible autoimmune response to an infection.


Children usually recover within 6-8 weeks. However, complications can develop if the condition is not diagnosed and treated promptly.


Complications of Kawasaki disease

Around a quarter of children with Kawasaki disease will experience heart complications because Kawasaki disease causes inflammation and swelling of blood vessels, which affects blood supply to the heart of a child.


Damage to the heart muscle and valves leads to heart abnormalities and these will be closely monitored by a cardiologist(heart specialist) and an appropriate treatment decided on if heart abnormalities have taken place as a result of Kawasaki disease.


Treatment

Diagnosis and treatment of Kawasaki disease must only be done in hospital. The sooner the condition is diagnosed and the treatment begins, the higher chances that there will be no or fewer complications from Kawasaki disease for a child.


Treatment usually involves aspirin and intravenous immunoglobulin(IVIG is a solution of antibodies injected into a vein). It is important not to self-diagnose or self-medicate, especially with Aspirin as it causes side effects including Reye’s syndrome characterised by liver and brain damage. Always seek medical help and do not self-diagnose and self-medicate.


Food for thought

As Kawasaki disease symptoms and the symptoms of a novel PMIS-TS(paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection) are similar, it is important to stay alert and seek medical help as soon as any signs or symptoms develop in our children.


As time proves, there is still a lot to learn about the SARS-CoV-2 virus causing COVID-19 and its effects in children.


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