Emmi Giacomo, Bettiol Alessandra, Hatemi Gülen, Prisco Domenico
Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy; Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, VIC, Australia.
Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Lancet. 2024 Mar 16;403(10431):1093-1108. doi: 10.1016/S0140-6736(23)02629-6. Epub 2024 Feb 22.
Behçet's syndrome is a rare, chronic multisystemic inflammatory disorder also known as the Silk Route disease due to its geographical distribution. Behçet's syndrome is a multifactorial disease and infectious, genetic, epigenetic, and immunological factors contribute to its pathogenesis. Its heterogeneous spectrum of clinical features include mucocutaneous, articular, ocular, vascular, neurological, and gastrointestinal manifestations that can present with a relapsing and remitting course. Differential diagnosis is often hampered by the non-specific clinical presentation and the absence of laboratory biomarkers or pathognomonic histological features. The therapeutic approach is tailored on the basis of patient-specific manifestations and relies on glucocorticoids, colchicine, and traditional and biological immunosuppressants. Despite progress in the knowledge and management of the disease, unmet needs in diagnostics, monitoring, prediction, and treatment personalisation challenge clinical practice, making Behçet's syndrome a complex disorder associated with an increased risk of morbidity.
白塞病是一种罕见的慢性多系统炎症性疾病，因其地理分布也被称为丝绸之路病。白塞病是一种多因素疾病，感染、遗传、表观遗传和免疫因素都参与其发病机制。其临床特征具有异质性，包括黏膜皮肤、关节、眼部、血管、神经和胃肠道表现，可呈复发-缓解病程。非特异性临床表现以及缺乏实验室生物标志物或特征性组织学表现常常妨碍鉴别诊断。治疗方法根据患者的具体表现进行调整，依赖于糖皮质激素、秋水仙碱以及传统和生物免疫抑制剂。尽管在该疾病的认识和管理方面取得了进展，但诊断、监测、预测和治疗个体化方面未满足的需求给临床实践带来了挑战，使白塞病成为一种与发病风险增加相关的复杂疾病。
