Multisystem inflammatory Syndrome

Multisystem inflammatory Syndrome

August 02, 2020

The Novel Coronavirus disease (COVID-19) Pandemic has been a steep learning curve for doctors, scientists and health care communities worldwide. New information especially with regards to children is surfacing which has led to significant anxiety in parents.

Children so far have fortunately not been very affected in large numbers by this illness. Internationally Children have comprised 1.7-2% of national cases. Most children have had mild symptoms and many instances have developed no symptoms at all despite testing positive.

There has been a lot of recent newspaper articles, case reports and social media reports of a severe Kawasaki like illness in children associated with this pandemic.

What is Multi inflammatory syndrome in children?

It is an inflammatory condition affecting heart, lungs, kidneys, brain, skin and gastrointestinal tract. The cause is unknown but is often seen post covid 19 infection in children. The condition may vary from mild to severe illness, often leading to death

Symptoms include fever, abdominal pain, diarrhea, vomiting, rash, neck pain, tiredness, blood shot eyes

Worsening of breathlessness, chest pain, confusion, sleepiness, bluish discoloration of face and lips, severe pain abdomen are conditions for immediate hospitalization

What is Kawasaki disease?

Kawasaki disease is a well-known autoimmune self-limiting vasculitis (the cells of your immune system attack your own body especially small and medium sized blood vessels including the vessels supplying the heart). The trigger of this autoimmune phenomenon remains a conundrum since it was first discovered In January 1967 in Tokyo and several theories including an infective trigger to super antigen have been explored. It affects more boys than girls and predominantly affects children under the age of 5 with an annual incidence of 10-100/100,000 children. The incidence of Kawasaki Disease has been increasing over the past decade which is partly attributed to a greater awareness and diagnosis.

There is no specific test to confirm whether the child has Kawasaki’s disease rather it is diagnosed based on a constellation of clinical symptoms, blood tests and scans like the echocardiogram of the heart.

The main features of Kawasaki disease are:

  • Fever for at least a period of 5 days
  • Erythema(redness) of lips and the mouth cavity or cracks on the lips
  • Rash on the body
  • Swelling and redness of hands and feet later with peeling of the skin tips of toes and fingers
  • Redness of the whites of the eyes(conjunctivitis)
  • Swelling of lymph nodes

The aim of treatment of Kawasaki disease is the to reduce the inflammation and eventually protect the heart. Your pediatrician along with input from a pediatric rheumatologist will suggest medication such as immunoglobulins, aspirin and/or steroids which will need to be done in the hospital.

Kawasaki like illness in the COVID-19 pandemic

Many countries especially Italy, France, Spain, USA and UK that have seen a peak of COVID-19 infection have reported a small number of cluster of children presenting very similar to Kawasaki disease in association with a toxic shock syndrome. This is seen nearly 3 weeks after the peak of the infection. The term used to describe it is ‘Multisystem inflammatory disorder of children and adolescents.

The key is the presentation of these children a couple of weeks after the peak of the infection in the general population (generally adults). Tests on these children show that the COVID-19 RT-PCR test may be negative and they may be positive for the antibodies. This enhances our understanding that it is not the acute infection with the virus but hyper inflammatory response of the body that leads to involvement of multiple organs including the heart. Many studies are currently underway to make us understand this new presentation of the COVID-19 that is affecting children.

Although there are some similarities with the Kawasaki disease that has been discussed above, it is an entirely new entity which requires urgent recognition and treatment.

The small number of children who have been affected with multi inflammatory syndrome are generally between 5-15 years of age and have presented with:

  • A fever for at least 3 days
  • Features resembling Kawasaki disease: rash, redness to the eyes, redness with swelling to the palms and soles or the mouth cavity. In addition to this they may have
  • loose stools, vomiting, tummy pain that may be severe,
  • low blood pressure
  • neck pain
  • Ability to affect multiple organs including the heart early on.
  • Breathing may or may not be affected

Although rare the multisystem inflammatory disorder requires urgent immediate medical attention which will enable your Pediatrician to examine and assess your child at the earliest, conduct the tests needed and commence treatment without delay.

Management will generally be hospitalization. The child after an initial assessment will have a treatment protocol drawn up which will involve medicines that will help dampen this inflammatory response. These will generally comprise use of steroids, immunoglobulins, antibiotics and immune-modulators to name a few. The encouraging news is that children who have presented with the multisystem inflammatory disorder have done extremely well when identified early.

It is important to reiterate here that this is extremely rare. The nature of the Multisystem Inflammatory syndrome in the COVID-19 pandemic will become clearer as we have more data from countries that have experienced a peak before us. The key for now is to be aware and vigilant but not to panic.

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