Abstract Number ‐ 282: Surgical Treatment for Moyamoya Disease with Coexisting Autoimmune Conditions: A Systematic Review

Abstract

Introduction There have been several studies of Moyamoya disease (MMD) with concurrent autoimmune (AI) disease. It has been proposed that there may be a causal or synergistic effect of autoimmune and inflammatory conditions in patients with MMD. As such, in the present study we seek to characterizethe surgical outcomes of adults and children with MMD and coexisting AI conditions. Methods A systematic review was performed of published 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 A total of 149 studies were included, encompassing 257 patients. Of the 257 patients with concurrent AI and MMD, 107 (88 females, 19 males) underwent surgery. The average age was 31.3 years (Range 5–65; SD 12.5). Five cases presented with hemorrhagic symptoms, 87 ischemic, and 15 were unspecified. The most commonly associated AI conditions were Graves’ disease (71 cases, 66.4%), Lupus (17 cases, 15.9%), and T1DM (6 cases, 5.6%).Follow‐up was reported in 25 cases and was an average of 17.8 months. Surgical outcomes were reported in 82 cases; 63 (76.8%) improved, 7 (8.5%) were stable, 9 (10.9%) developed a new deficit, and 3 (3.7%) died at most recent follow up. Eight patients (7.5%) had disease progression. 63 (58.8%) patients were treated with additional autoimmune therapy although this therapy did not lead to any difference in outcomes. Twenty patients (18.7%) required additional supportive medical therapy (eg: aspirin, statins). Conclusions Moyamoya disease may be multifactorial. Patients with concomitant AI and Moyamoya disease may be at increased risk and warrant additional surveillance or intervention. The ideal therapy for these patients remains to be understood. These findings warrant further study into optimal diagnostic and therapeutic techniques for concomitant MMD and AI conditions.