Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, 34098, Istanbul, Turkey.
Intern Emerg Med. 2019 Aug;14(5):661-675. doi: 10.1007/s11739-019-02035-1. Epub 2019 Jan 25.
Behçet's syndrome (BS) is a complex disease that shows important heterogeneity in clinical findings and physiopathology. Its treatment can be problematic as BS manifestations in different organs may respond differently to the same drug. The cornerstone of therapy for inducing remission is corticosteroids whereas immunomodulatory and immunosuppressive agents such as colchicine, azathioprine, cyclosporine-A, interferon-alpha, and cyclophosphamide are used as steroid-sparing agents and to prevent further relapses. However, a considerable number of patients continue to have mucocutaneous lesions despite therapy, and some patients require more aggressive treatment for refractory major organ involvement. Tumor necrosis factor alpha inhibitors, especially infliximab and adalimumab, are increasingly used for various refractory BS manifestations despite the lack of controlled studies. In this review, we aim to focus on both the traditional and new treatment modalities for BS, with more emphasis on recent data on newer agents.
贝赫切特综合征(BS)是一种复杂的疾病,其临床表现和病理生理学存在显著异质性。BS 的治疗可能存在问题,因为不同器官的 BS 表现可能对同一药物的反应不同。诱导缓解的治疗基石是皮质类固醇,而秋水仙碱、巯嘌呤、环孢素 A、干扰素-α和环磷酰胺等免疫调节剂和免疫抑制剂则被用作皮质类固醇的辅助药物,以预防进一步复发。然而,尽管进行了治疗,仍有相当数量的患者持续存在黏膜皮肤损伤,一些患者需要更积极的治疗以应对难治性主要器官受累。尽管缺乏对照研究,但肿瘤坏死因子-α抑制剂,尤其是英夫利昔单抗和阿达木单抗,越来越多地用于各种难治性 BS 表现。在这篇综述中,我们旨在重点关注 BS 的传统和新治疗方法,更多地关注最近关于新型药物的数据。