Priya R Soni1, Magali Noval Rivas1,2, Moshe Arditi3,4,5. 1. Division of Pediatric Infectious Diseases and Immunology; Burns and Allen Research Institute, Cedars-Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, CA, 90048, USA. 2. Department of Biomedical Sciences-Infectious and Immunologic Diseases Research Center (IIDRC), Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA. 3. Division of Pediatric Infectious Diseases and Immunology; Burns and Allen Research Institute, Cedars-Sinai Medical Center and David Geffen School of Medicine at UCLA, Los Angeles, CA, 90048, USA. moshe.arditi@cshs.org. 4. Department of Biomedical Sciences-Infectious and Immunologic Diseases Research Center (IIDRC), Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA. moshe.arditi@cshs.org. 5. Smidt Heart Institute, Cedars-Sinai Medical Center, Los Angeles, CA, 90048, USA. moshe.arditi@cshs.org.
Abstract
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. 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.
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. 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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Authors: Stefanie Marek-Iannucci; Asli B Ozdemir; Debbie Moreira; Angela C Gomez; Malcolm Lane; Rebecca A Porritt; Youngho Lee; Kenichi Shimada; Masanori Abe; Aleksandr Stotland; David Zemmour; Sarah Parker; Elsa Sanchez-Lopez; Jennifer Van Eyk; Roberta A Gottlieb; Michael C Fishbein; Michael Karin; Timothy R Crother; Magali Noval Rivas; Moshe Arditi Journal: JCI Insight Date: 2021-09-22
Authors: Stefanie Marek-Iannucci; Asli D Yildirim; Syed M Hamid; Asli B Ozdemir; Angela C Gomez; Begüm Kocatürk; Rebecca A Porritt; Michael C Fishbein; Takao Iwawaki; Magali Noval Rivas; Ebru Erbay; Moshe Arditi Journal: JCI Insight Date: 2022-03-22