Center for Vasculitis Care and Research, Orthopedic and Rheumatology Institute, Cleveland Clinic, Desk A50, 9500 Euclid Avenue, Cleveland Ohio 4415, USA.
Best Pract Res Clin Rheumatol. 2010 Jun;24(3):413-26. doi: 10.1016/j.berh.2009.12.003.
While primary angiitis of the central system (PACNS) remains a rare entity, the poor specificity of the available diagnostic tests and its multiple mimics create a major diagnostic challenge. Recently, there have been advances in understanding PACNS and differentiating it from its mimics. A recent breakthrough is the proposal of reversible cerebral vasoconstriction syndromes (RCVS) as a unifying concept for a group of disorders that highly mimics PACNS. RCVS are characterised by acute-onset, recurrent headaches, with or without additional neurologic events, with reversible vasoconstriction of the central nervous system (CNS) vasculatures, mimicking CNS vasculitis. RCVS are considered the most common mimics of PACNS. Advances in our understanding of RCVS have allowed for identification of patients previously confused with PACNS. The scope of this article focusses on the work-up, differential diagnosis and evaluation of PACNS, as well as a discussion of the secondary CNS vasculitides with emphasis on their clinical findings, diagnoses and treatment.
虽然中枢神经系统原发性血管炎（PACNS）仍然是一种罕见的疾病，但现有的诊断测试特异性差，且有多种类似疾病，这给诊断带来了重大挑战。最近，人们对 PACNS 的认识和将其与类似疾病区分开来取得了一些进展。最近的一个突破是提出可逆性脑动脉收缩综合征（RCVS）作为一组高度模拟 PACNS 的疾病的统一概念。RCVS 的特征是急性发作、反复发作的头痛，伴有或不伴有其他神经系统事件，伴有中枢神经系统（CNS）血管的可逆性收缩，类似于 CNS 血管炎。RCVS 被认为是 PACNS 最常见的类似疾病。我们对 RCVS 的认识的进步使得以前与 PACNS 混淆的患者得到了明确诊断。本文的重点是 PACNS 的检查、鉴别诊断和评估，以及讨论继发性中枢神经系统血管炎，重点介绍其临床发现、诊断和治疗。
