Moyamoya syndrome is associated with production of autoantibodies to a broad spectrum of self-antigens and correlated with disease phenotype (P3175)

Abstract

Abstract Moyamoya syndrome (MMS) is a chronic cerebrovascular disorder characterized by a slowly progressive occlusion of the internal carotid artery. Previous study suggested that immune disorder and autoantibody production may involve in the pathogenesis of this disease. In order to elucidate the correlation of the autoAbs with the disease phenotype and to identify possible biomarkers, we utilized an autoantigen microarray bearing 83 specificities to characterize IgG and IgM autoantibodies expressed in the sera of MMS patients (n=56) and healthy controls (HC, n=31). 13 IgG autoantibodies and 35 IgM autoantibodies were significantly increased in MMS patients compared with HC (p<0.05). The increased expression of anti-MPO, anti-MBP and anti-PR3 in MMS sera were further confirmed by ELISA. Cluster analysis identified 4 autoAb patterns including chromosome related (anti-centromere and histone), ECM related (anti-matrigel, laminin), RNA related (anti-Ro60 and SSA/SSB) and thyroid related (anti-thyroglobulin and TPO) antibodies which were increased in MMS and correlated with different clinical manifestations including course of disease, number of involved brain vessels and thyroid activity, etc. Furthermore, MMS sera exhibited positive reaction with the patient brain vessels by IHC staining. The above findings indicate that IgG and IgM autoAbs may play an important role in the pathogenesis of MMS and could be useful biomarkers for the prediction of the disease