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目的:探讨成年出血型烟雾病复发出血特点及其危险因素。方法:回顾性纳入2011年11月至2019年11月九江市第一人民医院收治的成年出血型烟雾病患者，分析其首次发病的临床特点以及复发出血的类型和部位，并对复发出血的危险因素进行Kaplan-Meier生存曲线分析及多变量Cox比例风险回归分析。结果:共纳入111例成年出血型烟雾病患者，年龄(48.6±11.2)岁(范围24～82岁)。中位随访时间为39个月，27例患者(24.3%)复发出血，复发出血主要位于大脑后部(18例，66.7%)。复发出血组年龄显著低于未复发组[(42.19±11.0)岁对(50.61±10.48)岁；n t＝3.5，n P＝0.001]，而合并颅内动脉瘤(29.6%对11.9%；n χ2＝4.73，n P＝0.03)以及首次出血位于后部(51.9%对28.6%；n χ2＝4.92，n P＝0.027)的患者比例显著高于未复发组。复发出血类型及部位与首次出血常常不一致。对复发出血位于后部的患者首次出血部位进行的分析显示，首次出血位于后部(11/38，28.9%)的患者比例显著高于首次出血位于前部(7/73，9.6%；n P＝0.008)。Kaplan-Meier生存曲线分析显示，合并颅内动脉瘤(n P＝0.006)和首次出血位于后部(n P＝0.001)的患者更易复发出血，而血运重建术能减少复发出血(n P＝0.028)。Cox比例风险回归分析显示，合并颅内动脉瘤[风险比(hazard ratio, n HR)2.32，95%可信区间(confidence interval, n CI)1.05～5.16；n P＝0.038]以及首次出血位于后部(n HR 2.93，95% n CI 1.31～6.54；n P＝0.009)是出血型烟雾病患者复发出血的独立危险因素。Kaplan-Meier生存曲线以及Cox多变量比例风险回归分析显示，合并颅内动脉瘤也是复发后部出血的独立危险因素(n HR 2.97，95% n CI 1.06～8.26；n P＝0.034)。n 结论:成年出血型烟雾病的首次出血与复发出血类型及部位常常不一致，首次出血位于后部以及合并颅内动脉瘤是复发出血的独立危险因素。“,”Objective:To investigate the characteristics of recurrent hemorrhage and its risk factors in adults patients with hemorrhagic moyamoya disease.Methods:Adult patients with hemorrhagic moyamoya disease admitted to Jiujiang No. 1 People's Hospital from November 2011 to November 2019 were enrolled retrospectively. The clinical characteristics of the first onset and the type and location of recurrent bleeding were analyzed. Kaplan-Meier survival curve analysis and multivariate Cox proportional hazard regression analysis were performed on the risk factors for recurrent bleeding.Results:A total of 111 adult patients with hemorrhagic moyamoya disease were enrolled, and their age was 48.6±11.2 years (range, 24-82 years). The median follow-up time was 39 months. Twenty-seven patients (24.3%) had recurrent bleeding, which was mainly located in the posterior cerebrum (n n=18, 66.7%). The age of the recurrent bleeding group was significantly lower than that of the non-recurrent bleeding group (42.19±11.0 years old n vs. 50.61±10.48 years old; n t=3.5, n P=0.001), and the proportion of patients with concurrent intracranial aneurysms (29.6% n vs. 11.9%; n χ2=4.73, n P=0.03) and whose first bleeding was located in the posterior cerebrum (51.9% n vs. 28.6%; n χ2=4.92, n P=0.027) were significantly higher than those in the non-recurrent bleeding group. The type and location of recurrent bleeding were often inconsistent with the first bleeding. Analysis of the location of the first bleeding in patients with recurrent bleeding in the posterior cerebrum showed that the proportion of patients with the first bleeding in the posterior cerebrum (11/38, 28.9%) was significantly higher than that in the anterior cerebrum (7/73, 9.6%; n P=0.008). Kaplan-Meier survival curve analysis showed that patients with concurrent intracranial aneurysm (n P=0.006) and first bleeding in the posterior cerebrum (n P=0.001) were more likely to relapse bleeding, and revascularization could reduce recurrent bleeding (n P=0.028). Cox proportional hazard regression analysis showed that concurrent intracranial aneurysm (hazard ratio [n HR] 2.32, 95% confidence interval [n CI]1.05-5.16; n P=0.038) and the first bleeding in the posterior cerebrum (n HR 2.93, 95% n CI 1.31-6.54; n P=0.009) were the independent risk factors for recurrent bleeding in patients with hemorrhagic moyamoya disease. Kaplan-Meier survival curve and Cox multivariate proportional hazard regression analysis showed that concurrent intracranial aneurysm was also an independent risk factor for recurrence of posterior hemorrhage (n HR 2.97, 95% n CI 1.06-8.26; n P=0.034).n Conclusion:The type and location of the first bleeding and recurrent bleeding in adults with hemorrhagic moyamoya disease were often inconsistent. The first bleeding in the posterior cerebrum and concurrent intracranial aneurysm were the independent risk factors for recurrent bleeding.