PMID- 32812215 OWN - NLM STAT- MEDLINE DCOM- 20210518 LR - 20221108 IS - 1365-2249 (Electronic) IS - 0009-9104 (Print) IS - 0009-9104 (Linking) VI - 202 IP - 3 DP - 2020 Dec TI - Characterization of circulating immune cells in acute Kawasaki disease suggests exposure to different antigens. PG - 263-272 LID - 10.1111/cei.13506 [doi] AB - Kawasaki disease (KD) is an acute pediatric vasculitis of unknown etiology that can cause coronary artery aneurysms, and is the leading cause of acquired heart disease in children. We studied aspects of the innate and adaptive immune response in 17 acute KD children prior to treatment with intravenous immunoglobulin. Distinct patterns within the innate immune response correlated with specific clinical features. Proinflammatory myeloid dendritic cells (mDC) were abundant in four of 17 (23.5%) subjects who were older and manifested severe inflammation with clinical myocarditis and elevated hepatobiliary enzyme levels. Of the nine subjects with low levels of anti-inflammatory, tolerogenic mDC, six had enlarged cervical lymph nodes at diagnosis. In contrast, the adaptive immune repertoire varied greatly with no discernible patterns or associations with clinical features. Two subjects with aneurysms had numerous circulating CD8(+) T cells. Ten subjects showed low CD4(+) T cell numbers and seven subjects had CD4(+) T cells in the normal range. CD4(+) T cells expressed interleukin-7 receptor (IL-7R), suggesting repeated antigenic stimulation. Thymic-derived regulatory T cells (nT(reg) ) and peripherally induced regulatory T cells (iT(reg) ) were also enumerated, with the majority having the nT(reg) phenotype. Natural killer (NK) and NK T cell numbers were similar across all subjects. Taken together, the results of the immune monitoring suggest that KD may have multiple triggers that stimulate different arms of the innate and adaptive compartment in KD patients. Thus, it is possible that diverse antigens may participate in the pathogenesis of KD. CI - (c) 2020 The Authors. Clinical & Experimental Immunology published by John Wiley & Sons Ltd on behalf of British Society for Immunology. FAU - Burns, J C AU - Burns JC AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Hsieh, L E AU - Hsieh LE AUID- ORCID: 0000-0003-1179-2229 AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Kumar, J AU - Kumar J AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Behnamfar, N AU - Behnamfar N AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Shimizu, C AU - Shimizu C AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Sivilay, N AU - Sivilay N AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Tremoulet, A H AU - Tremoulet AH AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. FAU - Franco, A AU - Franco A AD - School of Medicine, Department of Pediatrics, University of California San Diego, La Jolla, CA, USA. LA - eng GR - R01 AI143586/AI/NIAID NIH HHS/United States PT - Clinical Trial PT - Journal Article PT - Research Support, N.I.H., Extramural PT - Research Support, Non-U.S. Gov't DEP - 20200827 PL - England TA - Clin Exp Immunol JT - Clinical and experimental immunology JID - 0057202 RN - 0 (Antigens) RN - 0 (Immunoglobulins, Intravenous) SB - IM MH - Acute Disease MH - Antigens/*blood/immunology MH - Child MH - Child, Preschool MH - Dendritic Cells/immunology/*metabolism MH - Female MH - Humans MH - Immunoglobulins, Intravenous/administration & dosage MH - Infant MH - Lymphocytes/immunology/*metabolism MH - Male MH - Mucocutaneous Lymph Node Syndrome/*blood/drug therapy PMC - PMC7670149 OTO - NOTNLM OT - acute pediatric vasculitis OT - antigenic triggers OT - immune monitoring COIS- None of the authors have financial or commercial conflicts of interest. EDAT- 2020/08/20 06:00 MHDA- 2021/05/19 06:00 PMCR- 2020/08/27 CRDT- 2020/08/20 06:00 PHST- 2020/04/13 00:00 [received] PHST- 2020/08/03 00:00 [revised] PHST- 2020/08/04 00:00 [accepted] PHST- 2020/08/20 06:00 [pubmed] PHST- 2021/05/19 06:00 [medline] PHST- 2020/08/20 06:00 [entrez] PHST- 2020/08/27 00:00 [pmc-release] AID - CEI13506 [pii] AID - 10.1111/cei.13506 [doi] PST - ppublish SO - Clin Exp Immunol. 2020 Dec;202(3):263-272. doi: 10.1111/cei.13506. Epub 2020 Aug 27.