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

Jian-Bin Chen Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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Yi Liu Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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Liang-Xue Zhou Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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Hong Sun Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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Min He Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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Chao You Department of Neurosurgery, West China Hospital, West China School of Medicine, Sichuan University, Chengdu, Sichuan Province, People’s Republic of China

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OBJECT

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.

ABBREVIATIONS

AIG = autoimmune gastritis; APAS = antiphospholipid antibody syndrome; APS = autoimmune polyendocrine syndrome; GD = Graves’ disease; HT = Hashimoto thyroiditis; ICA = internal carotid artery; ICD-10 = International Classification of Diseases, 10th Revision; MCA= middle cerebral artery; MMD = moyamoya disease; MMV = moyamoya vasculopathy; PBC = primary biliary cirrhosis; SLE = systemic lupus erythematosus; TIA = transient ischemic attack.
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