Moyamoya and Inflammation.

BACKGROUND: More than 50 years have elapsed since moyamoya disease was initially described; however, the disease etiology remains unknown. Although certain genetic loci and immunologic characteristics are associated with moyamoya disease, this does not fully explain its pathophysiology. An association with inflammatory disease has been postulated but not rigorously explored. We sought to examine the epidemiologic association of moyamoya and inflammatory diseases by analyzing data from a large administrative database. METHODS: The National Inpatient Sample database for 2009-2012 was obtained. The diagnoses of moyamoya disease and inflammatory diseases were made using the International Classification of Disease, 9th revision. Sample prevalence, sex, age, and admission type were recorded. Patients were grouped into inflammatory disease clusters on the basis of the presence of diagnosis codes for atherosclerotic, adult-onset autoimmune, and juvenile-onset autoimmune diseases. RESULTS: There were 2633 total admissions for moyamoya disease. Atherosclerotic (P < 0.05) and juvenile-onset autoimmune disease (P < 0.05) were associated with moyamoya disease in both pediatric and adult patient groups. Adult-onset autoimmune disease was associated with moyamoya disease in pediatric (P < 0.05) but not adult groups. CONCLUSION: Moyamoya is associated with inflammatory disease clusters in both pediatric and adult populations. Further studies are warranted to investigate the pathophysiologic association between moyamoya disease and inflammatory disease processes.