The Clinical Features and Risk Factors of Parenchymal Neuro-Behcet’s Disease

• Main Authors: Dong Yan, JinJing Liu, Yuehua Zhang, Wei Yuan, Yan Xu, Jing Shi, Chaoran Li, Yining Wang, Linyi Peng, Yunjiao Yang, Jiaxin Zhou, Di Wu, Zhichun Liu, Xiaofeng Zeng, Fengchun Zhang, Wenjie Zheng, Yan Zhao
• Format: Article
• Language: English
• Published: Hindawi Limited 2019-01-01
• Series: Journal of Immunology Research
• Online Access: http://dx.doi.org/10.1155/2019/7371458

To investigate the clinical features of parenchymal neuro-Behcet’s disease (p-NBD), we retrospectively reviewed the medical records of 1009 BD patients admitted to Peking Union Medical College Hospital from 2000 to 2016. Forty-two patients (25 males and 17 females) with p-NBD and eighty-four age- and sex-matched BD patients without neurological involvement who were served as controls were enrolled. Neurological onset was concomitant with the onset of BD in six cases (14.3%). Pyramidal signs (50.0%) and headache (33.3%) were the most common manifestations. On MRI, the lesions were mainly in the midline structures and hyperintense in the T2-weighted image. The most common lesion was the brainstem (54.8%). Spinal cord involvement was observed in five cases, four of which with cervical cord involvement. Multifocal lesions were observed in 13 patients. Ocular involvement was more prevalent in p-NBD (35.7%) (P=0.041, OR=2.36, 95% CI=1.03-5.44) compared with controls. All patients received corticosteroids and immunosuppressants, mainly cyclophosphamide (39/42). Six patients with severe/refractory condition received biological agents and achieved response measured by decreased Rankin score (P=0.002). With a median follow-up of 28 months, 22 patients (61.1%) achieved clinical improvements, while 10 (27.8%) relapsed and 4 died (mortality rate 11.1%). p-NBD is a rare yet disabling and life-threatening complication of BD. Ocular involvement is a risk factor for p-NBD. Promptly aggressive treatment is essential for improving prognosis, and biological agents might be a promising approach for severe/refractory p-NBD.