133 - FROM KAWASAKI TO MULTI SYSTEM INFLAMMATORY DISEASE : LEVERAGING INSIGHTS IN PEDIATRIC VASCULITIS IN THE CREATION OF AN ALGORITHM TO IMPROVE DIAGNOSIS AND TREATMENT STRATEGIES

Background:

Multisystem inflammatory syndrome in children (MIS-C) is a rare condition which occurs 2 to 6 weeks after a child is infected with SARS CoV- 2. As per CDC, it affects 1 in 3000- 4000 children.

MIS-C is characterized by several multi systemic manifestations that resemble Kawasaki disease. Classic Kawasaki disease is characterized by fever for 5 days or more in addition to 4/5 clinical symptoms which include rash, palm/sole swelling, conjunctivitis, cervical lymphadenopathy, and red cracked lips slash strawberry tongue.

Methods:

At our institution we adapted a protocol based on existing protocols to assist physicians with the treatment for MIS-C.

The case definition included the following criteria:

Fever of more than 38° C for 5 days or more and 3 days or more (without a plausible cause) in addition to 2 of the following clinical symptoms which include headache, lymphadenopathy, sore throat, myalgia, irritability, conjunctivitis, oral mucosal changes, abdominal pain, vomiting, diarrhea, rash or swollen hands or feet.

Tier 1 lab testing includes SARS Co-V 2 PCR /antibody, complete blood count comprehensive metabolic panel, erythrocyte sedimentation rate and CRP. If abnormalities were noticed in tier 1 labs, tier 2 labs were obtained.

Tier 2 labs included Brain natriuretic peptide (BNP), troponin, procalcitonin, ferritin, lactate dehydrogenase, PT/PT, D- dimer, fibrinogen, triglycerides, urinalysis chest X-ray and EKG.

Based on clinical presentation, vital signs and laboratory findings, patients were admitted to pediatric hospital medicine team or intensive care unit.

Results:

We were able to successfully diagnose and treat roughly 60 patients with MIS-C though roughly 122 were admitted due to concern for the same. This is between 2020- 2022. Treatment included steroids, IVIG and Anakinra when required. Anakinra is a recombinant interleukin-1 receptor antagonist. When patients with elevated troponins and BNP were promptly started on Anakinra, cardiac outcome tremendously improved.

Conclusion:

MIS-C is a life-threatening manifestation of SARS CoV- 2 in children that needs prompt treatment. MIS-C shares similarities with Kawasaki disease as both involve an inflammatory cascade with predilection for the coronary arteries.

SARS-CoV-2 is a highly contagious virus with various systemic manifestations and detrimental sequelae. It is important for Pediatric providers to be familiar with diagnosis, complications, and treatment options. Anakinra successfully reduces systemic inflammatory response and should be promptly initiated when indicated.