A comprehensive update on Kawasaki disease vasculitis and myocarditis
PR Soni, M Noval Rivas, M Arditi - Current rheumatology reports, 2020 - Springer
PR Soni, M Noval Rivas, M Arditi
Current rheumatology reports, 2020•SpringerAbstract Purpose of the Review Kawasaki disease (KD) is a childhood systemic vasculitis of
unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can
result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD
children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel
therapeutic strategies. Here we review the latest findings in the field regarding specific
etiology, genetic associations, and advancements in treatment strategies to prevent …
unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can
result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD
children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel
therapeutic strategies. Here we review the latest findings in the field regarding specific
etiology, genetic associations, and advancements in treatment strategies to prevent …
Purpose of the Review
Kawasaki disease (KD) is a childhood systemic vasculitis of unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel therapeutic strategies. Here we review the latest findings in the field regarding specific etiology, genetic associations, and advancements in treatment strategies to prevent coronary aneurysms.
Recent Findings
Recent discoveries using the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model have accelerated the study of KD pathophysiology and have advanced treatment strategies including clinical trials for IL-1R antagonist, Anakinra.
Summary
KD remains an elusive pediatric vasculitis syndrome and is the leading cause of acquired heart disease among children in the USA and developed countries. Advancements in combination treatment for refractory KD with further understanding of novel genetic risk factors serve as a solid foundation for future research endeavors in the field.
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