Moyamoya angiopathy(MMA)is an intracranial large artery occlusive disease of unknown etiology,characterized by chronic stenosis/occlusion of the internal carotid artery system and the formation of abnormal vascular networks.Clinical and epidemiological studies have demonstrated that patients with MMA have a higher prevalence of immune-related diseases,suggesting that immune factors play an important role in the occurrence and progression of MMA.This paper aims to review the correlation between MMA and immune-related diseases such as infections,vasculitis,and autoimmune diseases,and explore the potential mechanisms of immune-related diseases involved in the pathogenesis of MMA.