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Open Access Case report

A novel autoantibody against ephrin type B receptor 2 in acute necrotizing encephalopathy

Tsuyoshi Shirai1, Hiroshi Fujii1*, Masao Ono2, Ryu Watanabe1, Yuko Shirota1, Shinichiro Saito1, Tomonori Ishii1, Masato Nose2 and Hideo Harigae1

Author Affiliations

1 Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai, Miyagi 980-8574, Japan

2 Department of Histopathology, Tohoku University Graduate School of Medicine, 1-1 Seiryo-cho, Aoba-ku, Sendai, Miyagi 980-8574, Japan

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Journal of Neuroinflammation 2013, 10:128  doi:10.1186/1742-2094-10-128

Published: 18 October 2013

Abstract

Acute necrotizing encephalopathy (ANE) is characterized by symmetrical brain necrosis, suggested to be due to breakdown of the blood–brain barrier (BBB). We experienced a rare case of ANE complicated with systemic lupus erythematosus (SLE), and found that the patient’s serum (V10-5) had binding activity to human umbilical vein endothelial cells (HUVECs). By SARF (Serological identification system for Autoantigens using a Retroviral vector and Flow cytometry) method using V10-5 IgG, a clone bound to V10-5 IgG was isolated. This cell clone was integrated with cDNA identical to EphB2, which plays critical roles in neuronal cells and endothelial cells. HUVECs and human brain microvascular endothelial cells expressed EphB2 and the V10-5 IgG bound specifically to EphB2-transfected cells. Anti-EphB2 antibody was not detected in other SLE patients without ANE. In this report, we identified EphB2 as a novel autoantigen, and anti-EphB2 antibody may define a novel group of brain disorders. Anti-EphB2 antibody can interfere not only with endothelial cells including those of the BBB (acting as an anti-endothelial cell antibody), but also neuronal cells (acting as an anti-neuronal antibody) if the BBB has been breached. Future studies should determine the clinical prevalence and specificity of anti-EphB2 antibody, and the molecular mechanisms by which anti-EphB2 antibody mediates neuronal and vascular pathological lesions.

Keywords:
Acute necrotizing encephalopathy; Autoantibody; Ephrin type B receptor 2; Serological identification system for Autoantigens using a Retroviral vector and Flow cytometry (SARF)