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

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.

doi：10.3969/j.issn.1672⁃6731.2021.06.014