Introduction Moyamoya disease was first described in the 1950’s. While six decades have passed, the underlying etiology of Moyamoya remains unknown. It has been postulated that underlying inflammatory or autoimmune (AI) processes may play a role in the development of this condition.
Objectives This systematic review aims to characterize the clinical and epidemiological attributes of concurrent moyamoya disease and AI conditions among the population.
Methods A systematic review was performed including studies reporting patient level data of Moyamoya disease and concomitant autoimmune disease. Relevant studies were identified using Preferred reporting Items for Systematic Reviews and Meta-Analysis criteria.
Results There were 739 results of which 149 were included in this study. The first reports of concurrent Moyamoya and autoimmune disease were in 1991 of 2 cases with associated Graves’ disease. They were treated with antithyroid medication alone and showed improvement. There was a total of 257 patients (192 females, 48 males, 17 unspecified). A total of 158 cases were reported as ‘Moyamoya Disease’ and 99 were reported as ‘Moyamoya Syndrome/Vasculopathy.’ Most cases were ischemic (175 ischemic, 15 hemorrhagic) in nature. Bilaterality of the disease was reported in 130 cases, unilaterality in 34 cases. The most commonly reported ethnicities were Chinese (26 cases), Japanese (17 cases), and Korean (17 cases). The most common AI conditions were Graves’ disease (150 cases, 58.4%), Lupus (26 cases, 10.1%), antiphospholipid syndrome (19 cases, 7.4%), Type 1 Diabetes (19 cases, 7.4%), and Multiple Sclerosis (7 cases, 2.7%).
Conclusions Numerous publications have reported Moyamoya with coexisting autoimmune conditions. The demographic characteristics align with the common autoimmune (female predominance) and Moyamoya (high east Asian prevalence) attributes. These results warrant further investigation into possible causal or synergistic mechanisms.
Disclosures D. Harrison: None. M. Bauman: None. N. Brown: None. R. Singh: None. J. Gendreau: None.
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