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Objective: To determine the effect of concurrent presence of atherosclerotic disease in other arterial distributions (ADOAD) on risk of 30 day stroke or death and ischemic stroke in the territory of the symptomatic intracranial artery beyond 30 days after enrollment in patients with severe (70-99%) intracranial stenosis recruited in the Stenting and Aggressive Medical Management for Preventing Recurrent Stroke in Intracranial Stenosis (SAMMPRIS) Trial.Methods: Patients with known history of myocardial infarction, angina, carotid endarterectomy, coronary artery bypass surgery, previous angioplasty and stenting, or peripheral arterial disease at baseline evaluation were classified as those with concurrent presence of ADOAD. Hazards ratio (HR) for primary event during follow up was estimated using Cox proportional hazards analysis after adjusting for age, gender, location of stenosis, and treatment allocated (stent placement versus best medical treatment).Results: A total of 126 of 451 patients included had concurrent presence of ADOAD. After adjusting for potential confounders, the risk of primary endpoint was not significantly different in patients with concurrent presence of ADOAD (HR 1.14, 95% confidence interval [CI] 0.7 - 1.9). The treatment effect of stent placement (compared with best medical treatment) was not different in patients with concurrent presence of ADOAD (HR of 0.9 and 1.1, respectively) after adjusting for potential cofounders. The risk of any ischemic stroke (HR 1.3, 95% CI 0.8 - 2.3) and death (HR 1.4, 95% CI 0.3 - 7.1) during follow up was not different with concurrent presence of ADOAD.Conclusions: There was no effect on cardiovascular endpoints in patients with high grade symptomatic intracranial stenosis and concurrent presence of ADOAD from both natural history and treatment perspective.