Department of Medicine, Section of Neuroscience, City University of New York School of Medicine, New York, NY; Department of Neurology, White Plains Hospital, White Plains, New York, USA.
Curr Opin Neurol. 2023 Dec 1;36(6):631-646. doi: 10.1097/WCO.0000000000001223. Epub 2023 Oct 12.
Vasculitis refers to heterogeneous clinicopathologic disorders that share the histopathology of inflammation of blood vessels. Unrecognized and therefore untreated, vasculitis of the nervous system or so called neurovasculitides, lead to pervasive injury and disability making these disorder of paramount importance to clinicians.
Headache is an important clue to vasculitic involvement of central nervous system (CNS) vessels. CNS vasculitis may be primary, in which only intracranial vessels are involved in the inflammatory process, or secondary to another known disorder with overlapping systemic involvement. A suspicion of vasculitis based on the history, clinical examination, or laboratory studies warrants prompt evaluation and treatment to forestall progression and avert cerebral ischemia or infarction. There has been remarkable progress in the pathogenesis, diagnosis, and treatment of primary adult and pediatric CNS vasculitides predicated on achievements in primary systemic forms.
Vasculitis can be diagnosed with certainty after intensive evaluation that includes tissue confirmation whenever possible. Clinicians must choose from among the available immune modulating, suppressive, and targeted immunotherapies to induce and maintain remission status and prevent relapse, tempered by the recognition of anticipated medication side effects.
脉管炎是指具有共同血管炎症组织病理学表现的异质性临床病理疾病。神经系统脉管炎，即所谓的神经脉管炎，如果未被识别和治疗，会导致广泛的损伤和残疾，这使得这些疾病对临床医生至关重要。
头痛是中枢神经系统（CNS）血管炎受累的重要线索。CNS 脉管炎可能是原发性的，仅颅内血管参与炎症过程，也可能是继发于另一种已知的伴有重叠全身受累的疾病。基于病史、临床检查或实验室研究怀疑脉管炎时，需要及时评估和治疗，以防止进展和避免脑缺血或梗死。基于原发性系统性疾病的成就，原发性成人和儿童 CNS 脉管炎的发病机制、诊断和治疗取得了显著进展。
在可能的情况下，通过强化评估（包括组织确认）可以明确诊断脉管炎。临床医生必须在现有的免疫调节、抑制和靶向免疫疗法中进行选择，以诱导和维持缓解状态，预防复发，并认识到预期的药物副作用。
