170 - KAWASAKI DISEASE IN CHILDREN WITH UNRELATED MEDICAL CONDITIONS: EXPLORING THE ASSOCIATION AND PATHOGENESIS

Kawasaki disease (KD) is the most common vasculitis in children below 5 years. The  etiology of KD  remains unknown although several hypotheses have been proposed. The most commonly accepted theory supports an infectious trigger leading to KD in a genetically predisposed individual.

Methods 

This study was based on a review of our hospital records from January 1994 to June 2023. During this period, we diagnosed 1350 patients with KD. Diagnosis of KD was based on standard criteria. The records of patients who had KD and associated unrelated medical conditions were evaluated.

Results:

Of the 1350 KD patients with KD, 10 had an associated unrelated medical condition. These included 3 children with Human Immunodeficiency virus infection, 3 children with nephrotic syndrome, 3 children with malignancy (2 had B cell -Acute Lymphoblastic Leukaemia ; 1 child had nesidioblastosis), 1 child with autoimmune hepatitis and 1 child with Periodic Fever Aphthous Stomatitis Pharyngitis and Adenitis (PFAPA). Out of the 10 children, 3 developed CAAs. Six children developed KD after the diagnosis of primary disease, whereas in 2 children diagnosis of KD was made at the time of presentation of primary illness. Two  children had had a KD like illness prior to the diagnosis of the unrelated illness. All children were treated with IVIg (2g/Kg)  and aspirin initially in high doses and later in antiplatelet doses. Recovery was uneventful. Patients with malignancy received chemotherapy while patients with HIV were started on anti retroviral therpay.

Conclusions:

KD can sometime occur in the context of an unrelated medical condition and this can be misleading. KD can be the first manifestation of the underlying illness or it can present later during the course of the illness. The treating paediatrician needs to have a high index of suspicion of KD whenever one encounters a child with fever for more than 5 days.