Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Hematology and Rheumatology, People's Hospital of Guangxi Zhuang Autonomous Region, Nanning, China.
Clin Rheumatol. 2018 Jan;37(1):51-57. doi: 10.1007/s10067-017-3718-2. Epub 2017 Jun 14.
The objective of the study is to investigate the clinical features of Behçet's disease (BD) complicated with cerebral venous sinus thrombosis (CVST). We retrospectively analyzed the clinical features of age- and gender-matched BD patients with and without CVST. Of the 840 BD patients enrolled, 21 (2.5%, 11 female, mean age 30.3 ± 9.5) were diagnosed as CVST. Eighteen patients (85.7%) developed neurologic manifestations after BD onset, with an average interval of 47.6 months (SD 36.1). Onset of neurologic symptoms was mostly progressive (89.5%), with headache (95.2%) being the most common symptom. Intracranial hypertension occurred in 17 cases (89.5%). Thrombosis was detected mostly in transverse (71.4%) and superior sagittal (66.7%) sinuses, with more frequently (66.7%) dual or multiple sinuses involved. In addition, extra cranial thrombosis was presented in 11 cases. Cerebral infarction was presented in four cases. Compared with BD patients without CVST, the prevalence of extra cranial vascular involvement (52.4 versus 20.6%; P = 0.005) and BDCAF 2006 score (3.7 ± 1.4 versus 1.8 ± 1.3; P = 1.7863 × 10) was higher in BD patients with CVST. During a median 7-month follow-up (range 0.3-88.8 months), 18 patients (85.7%) achieved remission following glucocorticoid, immunosuppressant therapy, anticoagulants, and dehydration, although 3 patients experienced (14.3%) flare. No death was reported. CVST is rare complication of BD and predominately progressively developed in active BD and after BD onset. It typically features intracranial hypertension syndrome and is frequently complicated with extra cranial thrombosis; thus, systemic thrombus screening is highly recommended. Promptly, diagnosis and aggressive treatment are keys to improve the prognosis.
研究目的在于探讨白塞病（BD）合并脑静脉窦血栓形成（CVST）的临床特征。我们回顾性分析了年龄和性别匹配的 BD 患者中伴有和不伴有 CVST 的临床特征。在纳入的 840 例 BD 患者中，21 例（2.5%，11 例女性，平均年龄 30.3±9.5）被诊断为 CVST。18 例（85.7%）在 BD 发病后出现神经系统表现，平均间隔时间为 47.6 个月（标准差 36.1）。神经系统症状的发作大多呈进行性（89.5%），最常见的症状是头痛（95.2%）。17 例（89.5%）发生颅内高压。血栓形成主要发生在横窦（71.4%）和上矢状窦（66.7%），更常见（66.7%）为双窦或多窦受累。此外，11 例患者还存在颅外血栓形成。4 例患者出现脑梗死。与无 CVST 的 BD 患者相比，CVST 组患者颅外血管受累的发生率（52.4%比 20.6%；P=0.005）和 BDCAF 2006 评分（3.7±1.4 比 1.8±1.3；P=1.7863×10）更高。在中位 7 个月的随访期（0.3-88.8 个月）内，18 例（85.7%）患者在接受糖皮质激素、免疫抑制剂、抗凝和脱水治疗后达到缓解，尽管 3 例患者（14.3%）出现复发。无死亡报告。CVST 是 BD 的罕见并发症，主要在活动期 BD 及发病后逐渐进展。它通常表现为颅内高压综合征，常并发颅外血栓形成，因此强烈建议进行全身血栓筛查。及时诊断和积极治疗是改善预后的关键。
