Shahram Farhad, Mæhlen Marthe T, Akhlaghi Massoomeh, Davatchi Fereydoun, Liao Yaping Joyce, Weyand Cornelia M
Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, USA.
Behcet's Disease Unit, Rheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Eur J Rheumatol. 2019 Jul 19;6(4):199-206. doi: 10.5152/eurjrheum.2019.18215. Print 2019 Oct.
Behçet's disease (BD) is a rare vasculitis that results in multi-organ inflammatory disease. At-risk populations are most prevalent in the Middle East and East Asia. Clinical data on BD in Western countries, especially in the United States, are scarce. We have compared clinical patterning of BD vasculitis in two geographically defined patient cohorts in the Western United States and Iran.
Comparative analysis of a retrospective cohort of 56 patients with BD evaluated at Stanford University Hospital between 2000 and 2016 and a cohort of 163 patients from the BD Registry at Tehran University of Medical Sciences. Clinical, demographic, laboratory, and treatment data were available. Comparisons were performed using descriptive statistics, Student's t-test, and χ-test.
The Stanford patients with BD were significantly younger at disease onset, had a higher proportion of females, and had longer disease duration than Iranian patients with BD. Genital ulcers, skin, joint, neurological, vascular, cardiopulmonary manifestations were all significantly more common in the Stanford cohort and 38% of Stanford patients had four or more organ systems involved compared with approximately 10% of Iranian patients. In contrast, Stanford patients had fewer ocular lesions (Stanford 21.4% vs. Iran 53.4% p<0.05), with the biggest difference seen for retinal vasculitis.
Patients with BD from the Western US have a more severe disease course when compared to Iranian patients with BD, as demonstrated by earlier onset and a higher rate of multi-organ involvement. The high risk of Iranian patients with BD developing vasculitis of ocular structures suggests distinct pathomechanisms driving ocular versus extra-ocular BD.
白塞病（BD）是一种罕见的血管炎，可导致多器官炎症性疾病。高危人群在中东和东亚最为普遍。西方国家，尤其是美国，关于白塞病的临床数据很少。我们比较了美国西部和伊朗两个地理区域定义的患者队列中白塞病血管炎的临床模式。
对2000年至2016年间在斯坦福大学医院评估的56例白塞病患者的回顾性队列和德黑兰医科大学白塞病登记处的163例患者队列进行比较分析。可获得临床、人口统计学、实验室和治疗数据。使用描述性统计、学生t检验和χ检验进行比较。
与伊朗白塞病患者相比，斯坦福大学的白塞病患者发病时年龄显著更小，女性比例更高，病程更长。生殖器溃疡、皮肤、关节、神经、血管、心肺表现在斯坦福队列中都明显更常见，38%的斯坦福患者有四个或更多器官系统受累，而伊朗患者约为10%。相比之下，斯坦福患者的眼部病变较少（斯坦福为21.4%，伊朗为53.4%，p<0.05），视网膜血管炎的差异最大。
与伊朗白塞病患者相比，美国西部的白塞病患者病程更严重，表现为发病更早和多器官受累率更高。伊朗白塞病患者发生眼部结构血管炎的高风险表明，驱动眼部与眼外白塞病的发病机制不同。
