Wei Yu-Zhen, Wang Hua-Bing, Yin Lin-Lin, Guo Ai, Zhang Lu-Lin, Sun Jia-Li, Lu Ping, Zhang Xing-Hu, Tian De-Cai
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
China National Clinical Research Center for Neurological Diseases, Beijing, China.
Front Neurol. 2025 Mar 25;16:1554989. doi: 10.3389/fneur.2025.1554989. eCollection 2025.
To assess the efficacy and safety of rituximab (RTX) in Chinese patients with primary angiitis of the central nervous system (PACNS).
Herein, we present the outcomes of 8 patients who received RTX for PACNS. Seven patients underwent a brain biopsy showing vasculitis, while the remaining patient had a digital subtraction angiogram and clinical findings highly suggestive of vasculitis. Clinical evaluation, laboratory tests, and imaging modalities were performed during the initial RTX administration and follow-up. The Expanded Disability Status Scale (EDSS) disability score was used to assess treatment response and degree of disability.
The median age at diagnosis of the 8 patients (2 females) was 37.0 years. All patients had active disease when RTX treatment was initiated. Five of the eight patients had refractory disease, and received one or more conventional immunosuppressants (IS). Three patients had contraindications or refused conventional IS. Patients were followed up until their death or the final follow-up visit (median 18 months; range: 0-40 months). The median EDSS score at the last visit (median 3.0; range 0-9.5) was lower than before RTX administration (median 6.5; range 1.5-9.5). In 6 patients, RTX administration was associated with a marked reduction in the number of flare-ups. Two of the six patients developed infections: one with pneumonia, and the other with tuberculosis. In one patient, parenchymal gadolinium enhancement persisted, and a new lesion was found following three courses of RTX.
Our data suggest that RTX therapy may be an additional treatment option for Chinese patients with PACNS.
评估利妥昔单抗（RTX）在中国原发性中枢神经系统血管炎（PACNS）患者中的疗效和安全性。
在此，我们报告了8例接受RTX治疗的PACNS患者的治疗结果。7例患者接受了脑活检，显示为血管炎，而其余1例患者进行了数字减影血管造影，临床检查结果高度提示血管炎。在首次RTX给药及随访期间进行了临床评估、实验室检查和影像学检查。采用扩展残疾状态量表（EDSS）残疾评分评估治疗反应和残疾程度。
8例患者（2例女性）诊断时的中位年龄为37.0岁。所有患者在开始RTX治疗时均患有活动性疾病。8例患者中有5例患有难治性疾病，并接受了一种或多种传统免疫抑制剂（IS）治疗。3例患者有使用传统IS的禁忌证或拒绝使用。对患者进行随访直至死亡或最后一次随访（中位时间18个月；范围：0 - 40个月）。末次随访时的中位EDSS评分（中位值3.0；范围0 - 9.5）低于RTX给药前（中位值6.5；范围1.5 - 9.5）。6例患者中，RTX给药与病情复发次数显著减少相关。6例患者中有2例发生感染：1例为肺炎，另1例为肺结核。1例患者脑实质钆增强持续存在，在接受三个疗程的RTX治疗后发现一个新病灶。
我们的数据表明，RTX治疗可能是中国PACNS患者的一种额外治疗选择。
