Hoshina Yoji, Delic Alen, Wong Ka-Ho, Lyden Stephanie, Kadish Robert, Smith Tammy L, Wright Melissa A, Shimura Daisuke, Clardy Stacey L
Department of Neurology, University of Utah, Salt Lake City, UT, USA.
George E. Wahlen Department of Veterans Affairs Medical Center, Salt Lake City, UT, USA.
Neurohospitalist. 2024 Apr;14(2):129-139. doi: 10.1177/19418744231223283. Epub 2023 Dec 18.
For the management of central nervous system (CNS) vasculitis, it is crucial to differentiate between primary and secondary CNS vasculitis and to understand the respective etiologies. We assessed the etiology, characteristics, and outcomes of patients with CNS vasculitis.
A single-center retrospective chart review was conducted at the University of Utah, Department of Neurology, between February 2011 and October 2022.
The median age of the 44 included patients at diagnosis was 54 years; 25.0% were men. Compared to primary CNS vasculitis, secondary CNS vasculitis exhibits higher fever incidence (observed in infectious and connective tissue disorder [CTD]-associated vasculitis), low glucose levels (mostly in infectious vasculitis) and unique cerebrospinal fluid oligoclonal bands (observed in infectious and CTD-associated vasculitis). Patients with inflammatory cerebral amyloid angiopathy (CAA) were older and more commonly had microhemorrhage than primary angiitis of the CNS (PACNS). All patients with CTD-associated vasculitis had a known history of CTD at presentation. Brain biopsies were performed on 10 of 17 PACNS patients and 4 of 8 inflammatory CAA patients, confirming vasculitis in 7 and 4 patients, respectively. Intravenous methylprednisolone was the predominant induction therapy (63.6%), and cyclophosphamide was the most used adjunctive therapy. Cyclophosphamide, rituximab, azathioprine, and mycophenolate mofetil were utilized as maintenance therapy, often with concurrent prednisone. Patients with inflammatory CAA had a higher tendency for relapse rates than PACNS.
This study highlights the variations in patients' characteristics, symptoms, and treatment for CNS vasculitis. Understanding these differences can lead to more efficient diagnostic and management strategies.
对于中枢神经系统（CNS）血管炎的管理，区分原发性和继发性CNS血管炎并了解各自的病因至关重要。我们评估了CNS血管炎患者的病因、特征及预后。
2011年2月至2022年10月期间，在犹他大学神经科进行了一项单中心回顾性病历审查。
纳入的44例患者诊断时的中位年龄为54岁；男性占25.0%。与原发性CNS血管炎相比，继发性CNS血管炎发热发生率更高（在感染性和结缔组织病[CTD]相关性血管炎中观察到）、血糖水平低（主要在感染性血管炎中）以及具有独特的脑脊液寡克隆带（在感染性和CTD相关性血管炎中观察到）。与中枢神经系统原发性血管炎（PACNS）相比，炎症性脑淀粉样血管病（CAA）患者年龄更大，微出血更为常见。所有CTD相关性血管炎患者在就诊时均有已知的CTD病史。17例PACNS患者中的10例和8例炎症性CAA患者中的4例进行了脑活检，分别证实7例和4例患者存在血管炎。静脉注射甲泼尼龙是主要的诱导治疗方法（63.6%），环磷酰胺是最常用的辅助治疗药物。环磷酰胺、利妥昔单抗、硫唑嘌呤和霉酚酸酯被用作维持治疗，通常同时使用泼尼松。炎症性CAA患者的复发率倾向高于PACNS。
本研究强调了CNS血管炎患者在特征、症状和治疗方面的差异。了解这些差异有助于制定更有效的诊断和管理策略。
