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The role of anti-endothelial cell antibodies in Kawasaki disease - in vitro and in vivo studies.

Abstract：

:Kawasaki disease (KD) is a systemic vasculitis with cardiac and noncardiac complications. Anti--endothelial cell antibodies (AECA) are found among many patients with KD. The aim of this study was to investigate the pathogenic role of AECA in KD using in vitro and in vivo experimental models. F(ab)2 fragments of IgG-AECA and IgM-AECA were affinity purified from a patient with active KD. Their endothelial binding and ability to induce a pro-adhesive and a pro-inflammatory phenotype were evaluated in vitro. Twenty Balb/C mice were immunized with KD-AECA or with control Ig (N-Ig) to induce AECA in a murine model by the idiotypic manipulation method. Both KD-AECA isotypes bind significantly to human umbilical vein endothelial cell (HUVEC) compared to N-Ig. The in vitro activity was demonstrated by the antibodies ability to activate endothelial cells resulting in increased IL-6 secretion, adhesion molecule expression and monocytic cell line (U937) adherence to HUVEC. Five of the mice that received KD-AECA developed murine AECA after 3 months. None of the mice that received N-Ig produced AECA. The murine AECA increased monocyte adhesion to EC in vitro, similarly to the AECA used for immunization. Furthermore, all the mice that developed AECA had proteinuria and IgG deposition in the renal mesangium. No histological or immunofluorescence evidence of cardiac vasculitis could be detected. AECA might play a role in the emergence of some of KD manifestations.

journal_name

Clin Exp Immunol

journal_title

Clinical and experimental immunology

authors

Grunebaum E,Blank M,Cohen S,Afek A,Kopolovic J,Meroni PL,Youinou P,Shoenfeld Y

doi

10.1046/j.1365-2249.2002.02000.x

subject

Has Abstract

pub_date

2002-11-01 00:00:00

pages

233-40

issue

2

eissn

0009-9104

issn

1365-2249

pii

2000

journal_volume

130

pub_type

杂志文章

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