Abstract Number ‐ 281: Clinical and Epidemiological Characteristics of Moyamoya Disease Presenting with Concomitant Autoimmune Conditions

Abstract

Introduction Moyamoya disease was first described in the 1950’s. After over six decades, however, the underlying etiology of Moyamoya still remains unknown. It has been postulated that underlying inflammatory or autoimmune (AI) processes may play a role in the development of this condition. In order to further investigate this hypothesis, we studied the clinical and epidemiological attributes of patients with concurrent Moyamoya disease and AI condition(s). 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 AI occurred in 1991 and involved 2 cases of Moyamoya and associated Graves’ disease. These patients were treated with antithyroid medication alone and showed improvement. Altogether, our search yielded 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 ethnicity was Chinese (26 cases), followed by Japanese (17 cases), and Korean (17 cases). The most common co‐presenting 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 There are numerous reports describing patients diagnosed with Moyamoya in addition to coexisting AI conditions. Our study shows that the demographic characteristics of co‐presentation align with the common autoimmune (female predominance) and Moyamoya (high east Asian prevalence) attributes. These results warrant further investigation into a possible causal or synergistic relationship between Moyamoya and AI disease.