中国现代神经疾病杂志 ›› 2021, Vol. 21 ›› Issue (6): 511-518. doi: 10.3969/j.issn.1672-6731.2021.06.014

• 临床研究 • 上一篇    下一篇

2 白塞病神经系统受累临床及免疫学特征分析

毛玉景, 刘田   

  1. 100044 北京大学人民医院风湿免疫科
  • 收稿日期:2021-05-15 出版日期:2021-06-25 发布日期:2021-06-26
  • 通讯作者: 刘田,Email:mikle317@163.com

Analysis of clinical and immunological characteristics of nervous system involvement in Behcet's disease

MAO Yu-jing, LIU Tian   

  1. Department of Rheumatology and Immunology, Peking University People's Hospital, Beijing 100044, China
  • Received:2021-05-15 Online:2021-06-25 Published:2021-06-26

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摘要：

目的 探讨神经白塞病（NBD）临床特点及免疫学类型，分析相关危险因素。方法 选择2013年1月至2019年1月经北京大学人民医院风湿免疫科确诊的神经白塞病患者共33例，采集其临床特征、各项免疫学指标（红细胞沉降率、C-反应蛋白、免疫球蛋白、补体C3和C4）以及影像学和脑脊液指标，并与非神经白塞病患者（41例）发病特点进行比较；采用单因素和多因素前进法Logistic回归分析神经白塞病危险因素。结果 与非神经白塞病患者相比，神经白塞病患者消化系统病变发生率（χ2=8.545，P=0.003）、血清C3（t=2.391，P=0.020）和C4（t=3.583，P=0.001）水平明显升高，其余各项指标组间差异无统计学意义（均P>0.05）。中枢神经系统实质型病变者占69.70%（23/33）、非实质型15.15%（5/33）、混合型6.06%（2/33），病变部位主要分布于脑干[18.18%（6/33）]、大脑半球[51.52%（17/33）]、小脑[9.09%（3/33）]或脑膜[12.12%（4/33）]；周围神经系统病变者仅占9.09%（3/33）。糖皮质激素为主要治疗药物，危重症可行激素冲击疗法（3例）或联合生物制剂治疗（5例）；除1例因颅内感染死亡，其余患者病情完全缓解21例、部分缓解7例、复发4例。Logistic回归分析，消化系统病变（OR=4.373，95% CI：1.309~14.614；P=0.017）和血清C4水平升高（OR=1.009，95% CI：1.000~1.018；P=0.043）为神经白塞病危险因素。结论 神经白塞病主要累及脑实质，临床表现复杂多样，激素联合免疫抑制剂或生物制剂治疗有效。临床表现为消化系统病变和血清补体C4水平升高的白塞病患者更易进展为神经白塞病。

关键词: 贝赫切特综合征, 神经系统, 变态反应和免疫学, 危险因素, Logistic模型

Abstract:

Objective To investigate the clinical features and risk factors of nervous system involvement in Behcet's disease (BD). Methods A total of 74 BD patients who were admitted to Peking University People's Hospital from January 2013 to January 2019 were selected and divided into neuro-Bechet's disaese (NBD) group for 33 cases and non-NBD group for 41 cases. Collect the clinical characteristics and immunological indicators of them[erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), immunoglobulin quantitative (IgG, IgA, IgM), complement (C3, C4) levels]. As well as imaging and cerebrospinal fluid indicators. Univariate Logistic regression analysis and multivariate Logistic regression analysis were used to analyze the risk factor of NBD. Results Compared with the non-NBD group, the incidence of digestive system lesions (χ2=8.545, P=0.003), the serum C3 (t=2.391, P=0.020) and C4 (t=3.583, P=0.001) were significantly increased, and there were no statistical significances in other indicators (P>0.05, for all). Among 33 cases of NBD, parenchymal-NBD (p-NBD) and non-parenchymal-NBD (np -NBD), and mixed were 69.70% (23/33), 15.15% (5/33) and 6.06% (2/33) respectively. And the lesions were mainly located in the brain stem[18.18% (6/33)], cerebral hemisphere[51.52% (17/33)], cerebellum[9.09% (3/33)] or meninges[12.12% (4/33)]. Peripheral nervous system diseases accounted for only 9.09% (3/33). All 33 cases were treated with glucocorticoids, 3 patients in the acute phase were also treated with methylprednisolone pulse therapy, and 5 patients used biologics. Except for one case died due to intracranial infection, 21 cases had complete remission, 7 cases had partial remission, and 4 cases relapsed. Logistic regression analysis showed that digestive system disease (OR=4.373, 95%CI:1.309-14.614; P=0.017) and increased C4 level (OR=1.009, 95%CI:1.000-1.018; P=0.043) were risk factors for NBD. Conclusions NBD mainly affects the brain parenchyma, and the clinical manifestations are complex and diverse. Aggressive use of glucocorticoids combined with immunosuppressive agents or biologics is necessary. BD patients with digestive system involvement or increased C4 level need to be more alert to the occurrence of NBD.

Key words: Behcet syndrome, Nervous system, Allergy and Immunology, Risk factors, Logistic models

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