Han Dan, Ma Bo, Liu Peng, Luo Guogang, Song Wenfeng
1 Department of Cardiology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
3 Department of Ophthalmology, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, China.
J Int Med Res. 2019 Jun;47(6):2694-2701. doi: 10.1177/0300060519839522. Epub 2019 Apr 16.
Behçet's syndrome (BS) is an idiopathic, chronic, relapsing, multisystem, vascular-inflammatory disease. Neuro-Behçet's syndrome (NBS) is a subtype of BS that mainly involves the central nervous system. Because of the heterogeneous involvement of NBS and the limited yearly numbers of new cases of NBS, estimating its course, prognosis, and treatment effect is difficult. Therefore, the efficacy of treatment for any form of NBS is unclear. We experienced a male patient with NBS and severe right uveitis. He received high-dose intravenous methylprednisolone (1000 mg/day) pulse therapy for 3 days. Intravenous injection of cyclophosphamide (400 mg, twice a day) and oral administration of prednisone (40 mg in the morning, 20 mg in the evening) were applied. Uveitis was treated with retrobulbar injection of triamcinolone acetonide (40 mg per week). The patient's response was rapid with improved symptoms and signs. The therapeutic regimen of patients with BS has greatly advanced, leading to evidence-based guidelines. Methylprednisolone pulse therapy is important in treatment of BS. Novel therapeutic options are currently being examined to improve the prognosis of BS. These efforts will undoubtedly shed new light on this complex syndrome.
白塞病（BS）是一种特发性、慢性、复发性、多系统血管炎性疾病。神经白塞病（NBS）是白塞病的一种亚型，主要累及中枢神经系统。由于NBS受累情况的异质性以及每年新发病例数量有限，难以估计其病程、预后及治疗效果。因此，任何形式的NBS的治疗效果尚不清楚。我们遇到一名患有NBS和严重右眼葡萄膜炎的男性患者。他接受了3天的大剂量静脉注射甲泼尼龙（1000毫克/天）冲击治疗。静脉注射环磷酰胺（400毫克，每日两次），并口服泼尼松（早上40毫克，晚上20毫克）。葡萄膜炎采用球后注射曲安奈德（每周40毫克）进行治疗。患者反应迅速，症状和体征均有改善。白塞病患者的治疗方案有了很大进展，形成了循证指南。甲泼尼龙冲击治疗在白塞病治疗中很重要。目前正在研究新的治疗选择以改善白塞病的预后。这些努力无疑将为这一复杂综合征带来新的曙光。
