Macit Betul, Esatoglu Sinem Nihal, Akyuz-Yesilyurt Kevser, Hatemi Gulen
Department of Dermatology, The Warren Alpert Medical School of Brown University, Providence, RI, USA.
Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Türkiye; Behçet's Disease Research Center, Istanbul University-Cerrahpasa, Istanbul, Türkiye.
Semin Arthritis Rheum. 2025 Aug;73:152744. doi: 10.1016/j.semarthrit.2025.152744. Epub 2025 May 8.
Behçet's syndrome (BS) is a multisystem vasculitis, and distinct clinical phenotypes with clustering of certain organ manifestations were proposed. However, studies from different cohorts have shown variability in the defined phenotypes. This was attributed to geographic and ethnic differences, but different studies from the same country have also shown variability in phenotype clusters. We aimed to explore the variability in clinical phenotype clustering across different countries and cohorts and possible reasons for these.
An electronic search was carried out in PubMed, EMBASE, and Cochrane Library for studies that assessed phenotype clusters in BS cohorts. Two reviewers independently performed the screening of titles, abstracts, and full texts using Covidence.
A total of 15 studies that assessed 17 different cohorts were identified. Several differences were identified in the clusters that were reported in these cohorts. Factors that were identified by this systematic review as possible causes of these differences were study design, statistical analysis method (hierarchical cluster analysis vs. factor analysis), patient population (pediatric vs. adult), setting, diagnostic/classification criteria (International Study Group vs. International Criteria for Behçet's Disease), disease duration, the definition of organ involvement (such as including cerebral sinus thrombosis in nervous system or vascular involvement), ascertainment of manifestations (such as gastrointestinal involvement confirmed by endoscopy or not), and time component for clustering of manifestations.
There is important variability in the phenotype clusters that are reported in different studies and this variability seems to stem from methodologic differences between the studies.
白塞病（BS）是一种多系统血管炎，有人提出了具有特定器官表现聚集的不同临床表型。然而，来自不同队列的研究显示，所定义的表型存在变异性。这归因于地理和种族差异，但来自同一国家的不同研究也显示出表型簇的变异性。我们旨在探讨不同国家和队列中临床表型簇的变异性及其可能原因。
在PubMed、EMBASE和Cochrane图书馆进行电子检索，以查找评估白塞病队列中表型簇的研究。两名审稿人使用Covidence独立进行标题、摘要和全文筛选。
共确定了15项评估17个不同队列的研究。在这些队列报告的簇中发现了几个差异。该系统评价确定的可能导致这些差异的因素包括研究设计、统计分析方法（层次聚类分析与因子分析）、患者群体（儿科与成人）、环境、诊断/分类标准（国际研究组与白塞病国际标准）、疾病持续时间、器官受累的定义（如神经系统是否包括脑静脉窦血栓形成或血管受累）、表现的确定（如胃肠道受累是否经内镜证实）以及表现聚类的时间因素。
不同研究报告的表型簇存在重要变异性，这种变异性似乎源于研究之间的方法学差异。
