Zhao Chao, Li Chuan, Duan Feng-Ju, Yan Qi, Zhang Zhuo, Du Ying, Zhang Wei
Department of Neurology, Tangdu Hospital, Fourth Military Medical University, Xi'an, China.
Front Neurol. 2021 Apr 15;12:595984. doi: 10.3389/fneur.2021.595984. eCollection 2021.
Neuro Behçet's disease (NBD) is a rare but most aggressive manifestation of Behçet's disease (BD) with a poor prognosis, and some patients even present a relapsing and treatment-resistant progressive course. In some relapsing NBD cases, traditional corticosteroids and immunosuppressive drugs show limited efficacy, while benefits of biological agents, such as anti-B-lymphocyte CD20 biological agent rituximab (RTX), gradually represent potential therapeutic advantages with clinical rapid remission and long-time maintenance. However, up to now, the optimal dosage of RTX in NBD is still elucidated. Here, we report two patients with relapsing NBD, despite continuous high dose steroids and sufficient azathioprine treatment, still presenting severe and relapsing meningoencephalitis or brainstem involvement. Repeated low-dose RTX (100 mg × 3/1 week apart, 100 mg repeated every 6 months) is then attempted with rapid recovery and sustained remission. The approach in our cases may expand therapeutic options and provide helpful references for relapsing NBD treatment.
神经白塞病（NBD）是白塞病（BD）一种罕见但最具侵袭性的表现形式，预后较差，一些患者甚至呈现复发且治疗抵抗的进展过程。在一些复发的NBD病例中，传统的皮质类固醇和免疫抑制药物疗效有限，而生物制剂，如抗B淋巴细胞CD20生物制剂利妥昔单抗（RTX），随着临床快速缓解和长期维持，逐渐展现出潜在的治疗优势。然而，截至目前，NBD中RTX的最佳剂量仍未明确。在此，我们报告两例复发的NBD患者，尽管持续接受高剂量类固醇和充足的硫唑嘌呤治疗，仍出现严重且复发的脑膜脑炎或脑干受累。随后尝试重复低剂量RTX（100 mg，间隔1周给药3次，每6个月重复100 mg），患者迅速康复并持续缓解。我们病例中的治疗方法可能会扩大治疗选择范围，并为复发NBD的治疗提供有益参考。
