Increased prevalence of autoimmune disease in patients with unilateral compared with bilateral moyamoya disease

Abstract

M oyamoya vasculopathy (MMV) is a disorder that is characterized by stenosis or occlusion of the terminal internal carotid artery (ICA) and the proximal portion of the anterior and/or middle cerebral arteries. Additionally, it is accompanied by net-like collateral vessel formations, which are termed the "moyamoya" vessels.14,18 Moyamoya disease (MMD) is defined by the presence of bilateral MMV with an unknown etiology or the presence of unilateral MMV at 1 side with obvious terminal contralateral ICA stenosis.4 Unilateral MMD is defined by typical angiographic evidence of MMD unilaterally with normal or equivocal contralateral findings. 4The concept that unilateral MMD is an early form of bilateral MMD remains controversial, and the pathogenesis of moyamoya disorders is not fully understood. 7,11,14,18 MMD has been reportedly associated with various entities. 2,8,9,13,16,24 Analysis of the coexisting diseases that occur with moyamoya may be helpful in exploring moyamoya pathogeneses. Several reports have noted MMD in association with autoimmune diseases, including autoim- obJective This study explored whether there were differences between the autoimmune disease prevalence rates in unilateral and bilateral moyamoya disease (MMD). methods The authors performed a retrospective review of data obtained from the medical records of their hospital, analyzing and comparing the clinical characteristics and prevalence rates of all autoimmune diseases that were associated with unilateral and bilateral MMD in their hospital from January 1995 to October 2014. results Three hundred sixteen patients with bilateral MMD and 68 with unilateral MMD were identified. The results indicated that patients with unilateral MMD were more likely to be female than were patients with bilateral MMD (67.6% vs 51.3%, p = 0.014, odds ratio [OR] 1.99). Overall, non-autoimmune comorbidities tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (17.6% vs 9.8%, p = 0.063, OR 1.97, chi-square test). Autoimmune thyroid disease and other autoimmune diseases also tended to be more prevalent in the unilateral MMD cases than in the bilateral MMD cases (19.1% vs 10.8%, p = 0.056, OR 1.96 and 8.8% vs 3.5%, p = 0.092, OR 2.77, respectively, chi-square test). The overall autoimmune disease prevalence in the unilateral MMD cases was significantly higher than in the bilateral MMD cases (26.5% vs 13.6%, p = 0.008, OR 2.29, 95% CI 1.22-4.28, chi-square test). Multiple logistic regression analysis showed that autoimmune disease was more likely to be associated with unilateral than with bilateral MMD (p = 0.039, OR 10.91, 95% CI 1. 13-105.25). coNclusioNs This study indicated a higher overall autoimmune disease prevalence in unilateral than in bilateral MMD. Unilateral MMD may be more associated with autoimmune disease than bilateral MMD. Different pathogenetic mechanisms may underlie moyamoya vessel formation in unilateral and bilateral MMD.

Association of moyamoya disease with thyroid autoantibodies and thyroid function

“…Secondly, the observation that in both our case series and the clinical study by Lei et al there was an association between the risk of moyamoya and the level of serum autoantibodies but not thyroid dysfunction suggests that autoimmunity is the factor in play as opposed to the function of the gland. This is in accordance with the result of the study of Chen et al [3]. Furthermore, considering the known regional differences in moyamoya [4], the finding of an association that is valid across different ethnicities indirectly suggests that the result of Lei et al deserves particular clinical and scientific attention.…”

Identification of HLA-DRB1*04:10 allele as risk allele for Japanese moyamoya disease and its association with autoimmune thyroid disease: A case-control study

“…HLA-DRB1*04 : 10 -positive patients account for ~10% of Japanese MMD patients; therefore, its involvement should be investigated in a larger population or other institutions. In addition, association between thyroid dysfunction and MMD is different between Asians and Caucasians: GD is more prevalent among Japanese MMD patients, whereas HT is more prevalent among Caucasian MMD patients [5–10]. The patients and controls in this study were residents of Tohoku and Kanto districts.…”

Moyamoya disease associated with ankylosing spondylitis in a 9-year-old child: a case report

“…Suzuki and Takaku suspected that the autoimmune played an important role in MMD [13]. Recently, more and more reports have showed the association between moyamoya and other autoimmune diseases, including Graves disease, Type 1 diabetes mellitus, systemic lupus erythematosus and Behcet's disease [14,15]. Review of the literatures reveals no report for MMD associate with AS.…”