title: Pediatric Kawasaki Disease and Adult Human Immunodeficiency Virus Kawasaki-Like Syndrome Are Likely the Same Malady.
abstract: Background.Pediatric Kawasaki disease (KD) and human immunodeficiency virus (HIV)+adult Kawasaki-like syndrome (KLS) are dramatic vasculitides with similar physical findings. Both syndromes include unusual arterial histopathology with immunoglobulin (Ig)A+plasma cells, and both impressively respond to pooled Ig therapy. Their distinctive presentations, histopathology, and therapeutic response suggest a common etiology. Because blood is in immediate contact with inflamed arteries, we investigated whether KD and KLS share an inflammatory signature in serum.Methods.A custom multiplex enzyme-linked immunosorbent assay (ELISA) defined the serum cytokine milieu in 2 adults with KLS during acute and convalescent phases, with asymptomatic HIV+subjects not taking antiretroviral therapy serving as controls. We then prospectively collected serum and plasma samples from children hospitalized with KD, unrelated febrile illnesses, and noninfectious conditions, analyzing them with a custom multiplex ELISA based on the KLS data.Results.Patients with KLS and KD subjects shared an inflammatory signature including acute-phase reactants reflecting tumor necrosis factor (TNF)-α biologic activity (soluble TNF receptor I/II) and endothelial/smooth muscle chemokinesCcl1(Th2),Ccl2(vascular inflammation), andCxcl11(plasma cell recruitment).Ccl1was specifically elevated in KD versus febrile controls, suggesting a unique relationship betweenCcl1and KD/KLS pathogenesis.Conclusions.This study defines a KD/KLS inflammatory signature mirroring a dysfunctional response likely to a common etiologic agent. The KD/KLS inflammatory signature based on elevated acute-phase reactants and specific endothelial/smooth muscle chemokines was able to identify KD subjects versus febrile controls, and it may serve as a practicable diagnostic test for KD.
authors.affiliation: Section of Infectious Diseases.
authors.forename: Raymond M
authors.initials: RM
authors.lastname: Johnson
authors.affiliation: Department of Pediatric Emergency Medicine , Children's Hospitals and Clinics of Minnesota , Minneapolis.
authors.forename: Kelly R
authors.initials: KR
authors.lastname: Bergmann
authors.affiliation: Ryan White Center for Pediatric Infectious Diseases and Global Health.
authors.forename: John J
authors.initials: JJ
authors.lastname: Manaloor
authors.affiliation: Biostatistics , Yale University School of Medicine , New Haven, Connecticut.
authors.forename: Xiaoqing
authors.initials: X
authors.lastname: Yu
authors.affiliation: Biostatistics , Indiana University School of Medicine , Indianapolis.
authors.forename: James E
authors.initials: JE
authors.lastname: Slaven
authors.affiliation: Department of Pediatric Emergency Medicine , Children's Hospitals and Clinics of Minnesota , Minneapolis.
authors.forename: Anupam B
authors.initials: AB
authors.lastname: Kharbanda
keywords: KD; KLS; Kawasaki disease; Kawasaki-like syndrome