Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Department of Neurosciences, Psychology, Drug Research and Child Health (NEUROFARBA), University of Florence, Florence, Italy.
Front Immunol. 2019 Dec 6;10:2830. doi: 10.3389/fimmu.2019.02830. eCollection 2019.
Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.
白塞病（BS）是一种多系统血管炎，其特征是不同的临床受累，包括黏膜皮肤、眼部、血管、神经和胃肠道表现。基于这种异质性，BS 很难被认为是单一的临床实体。越来越多的证据支持，在 BS 中，可以区分不同的表型，其特征是存在簇状共存的受累。即，已经报道了三种主要的 BS 表型：(a)黏膜皮肤和关节表型，(b) 实质外神经和周围血管表型，和 (c) 实质神经和眼部表型。迄今为止，BS 的管理指南一直集中在对每种特定 BS 表现的药物治疗上。然而，根据患者的特定表型而非单一疾病表现来调整治疗，可能是 BS 个体化治疗方法的有效策略。在本文献综述中，我们总结了目前关于主要 BS 表型一线、二线和三线治疗的药物治疗的证据。
