Nokhatha Shamma Ahmad Al, Aljaberi Najla, Zeyoudi Jawaher Al, Almarzooqi Ahlam, Soukieh Farah, Khan Mumtaz
Rheumatology Division, Department of Rheumatology, Tawam Hospital, Al Ain, United Arab Emirates.
Internal Medicine Department, College of Medicine and Health Sciences, UAE University, Al Ain, United Arab Emirates.
Rheumatol Int. 2025 May 11;45(6):140. doi: 10.1007/s00296-025-05892-6.
Behçet's disease (BD) is a rare multisystem vasculitis. Globally, BD exhibits a unique geographic distribution, with the highest prevalence along the historic Silk Road. To study the demographics, clinical characteristics, and outcomes of BD in the United Arab Emirates (UAE). A multicenter retrospective chart review study was conducted at hospitals in the UAE from 2008 to 2024. All adult and pediatric patients who were diagnosed with BD by a rheumatologist and treated within the timeframe were included in the study while those who received any alternative final diagnosis were excluded. The results were analyzed using descriptive and inferential statistics. One hundred and twenty-three patients were treated and monitored for BD. Most were female (n = 70, 57%). Their median age was 32 years (24-41), and approximately two-thirds were Emirati (n = 82, 67%). Twelve patients (10%) had a family history of BD, including 7 (6%) with first-degree BD. The time from the initial symptoms to diagnosis was 11.9 months (2.3-31.0). The most common clinical manifestations were oral ulcers (n = 99, 81%), arthralgia (n = 63, 51%), genital ulcers (n = 62, 50%), and ocular involvement (n = 37, 30%). Most of the patients were HLA-B51 positive (n = 92, 84%). At diagnosis, 36 (29%) met the ISG criteria, 73 (59%) met the ICBD criteria, and 50 (41%) met neither criteria. The male patients exhibited more major organ involvement (ocular, neurological, vascular, and cardiac), greater use of corticosteroids and anti-TNFa medications, and greater disease activity than the female patients. Among the patients with isolated ocular manifestations (n = 9) who met neither classification criteria, most were female (56%), with a median age of 37 years (26.5-3.5). None had a family history of autoimmune conditions, and all were HLA-B51 positive. Most of these patients (n = 8, 89%) received corticosteroids, 5 (56%) received azathioprine, and 2 (22%) received anti-TNFa therapies. These findings provide insights into the clinical profile of BD in the UAE. Notably, we found a high proportion of patients with positive HLA-B51 and ocular manifestation. Further research with larger sample sizes is needed to better understand these associations.
白塞病（BD）是一种罕见的多系统血管炎。在全球范围内，BD呈现出独特的地理分布，在历史上的丝绸之路沿线患病率最高。为了研究阿拉伯联合酋长国（阿联酋）BD的人口统计学、临床特征和结局。于2008年至2024年在阿联酋的医院开展了一项多中心回顾性病历审查研究。所有在该时间段内由风湿病学家诊断为BD并接受治疗的成年和儿科患者均纳入研究，而那些接受任何其他最终诊断的患者则被排除。使用描述性和推断性统计方法对结果进行分析。123例患者接受了BD治疗和监测。大多数为女性（n = 70，57%）。她们的中位年龄为32岁（24 - 41岁），约三分之二为阿联酋人（n = 82，67%）。12例患者（10%）有BD家族史，其中7例（6%）有一级亲属患BD。从初始症状到诊断的时间为11.9个月（2.3 - 31.0）。最常见的临床表现为口腔溃疡（n = 99，81%）、关节痛（n = 63，51%）、生殖器溃疡（n = 62，50%）和眼部受累（n = 37，30%）。大多数患者HLA - B51阳性（n = 92，84%）。在诊断时，36例（29%）符合国际白塞病研究组（ISG）标准，73例（59%）符合国际白塞病分类委员会（ICBD）标准，50例（41%）均不符合这两个标准。男性患者比女性患者表现出更多的主要器官受累（眼部、神经、血管和心脏），更多地使用皮质类固醇和抗TNFα药物，且疾病活动度更高。在9例既不符合分类标准又有孤立眼部表现的患者中，大多数为女性（56%），中位年龄为37岁（26.5 - 3.5）。均无自身免疫性疾病家族史，且均为HLA - B51阳性。这些患者中的大多数（n = 8，89%）接受了皮质类固醇治疗，5例（占56%）接受了硫唑嘌呤治疗，2例（占22%）接受了抗TNFα治疗。这些发现为阿联酋BD的临床特征提供了见解。值得注意的是，我们发现HLA - B51阳性和眼部表现的患者比例较高。需要更大样本量的进一步研究以更好地理解这些关联。
