Sundaram Soumya, Sylaja P N
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
Ann Indian Acad Neurol. 2022 Nov-Dec;25(6):1009-1018. doi: 10.4103/aian.aian_368_22. Epub 2022 Aug 10.
Primary angiitis of central nervous system (PACNS) is a rare idiopathic disorder affecting blood vessels of brain, spinal cord, and meninges, consequently leading to infarct and less frequently hemorrhage. CNS vasculitis can also occur as part of systemic vasculitis or secondary to autoimmune diseases or infections. The clinical manifestations of PACNS are non-specific and no single laboratory investigation or neuroimaging finding can reliably diagnose this condition. Histopathological evidence of transmural inflammation of blood vessels of CNS is the gold standard, but is generally pursued subsequent to conventional angiogram (CA) because of its invasive nature. The differentials of PACNS are exhaustive and include systemic vasculitis, secondary vasculitis, non-inflammatory intracranial vasculopathies, demyelination, and neoplasm. These alternative conditions can often be distinguished by history, examination, immunological testing, cerebrospinal fluid analysis, and neuroimaging. CA can detect vasculitic changes in the large to medium cerebral arteries but the specificity is low. Recent advancements in vessel wall imaging techniques have further enabled the distinction of various intracranial vasculopathies from CNS vasculitis. The disease has considerable morbidity and fatality unless timely treatment with immunosuppressive agents is initiated. Induction therapy with glucocorticoids and cyclophosphamide followed by azathioprine, mycophenolate mofetil, or methotrexate as maintenance therapy is the cornerstone of management. Biological agents such as rituximab and anti-tumour necrosis factor alpha inhibitors (infliximab and etanercept) may be used in refractory cases. This review discusses the approach to the diagnosis, determinants of outcome, and management.
中枢神经系统原发性血管炎（PACNS）是一种罕见的特发性疾病，可影响脑、脊髓和脑膜的血管，进而导致梗死，出血则较少见。中枢神经系统血管炎也可作为系统性血管炎的一部分出现，或继发于自身免疫性疾病或感染。PACNS的临床表现不具有特异性，没有单一的实验室检查或神经影像学检查结果能够可靠地诊断此病。中枢神经系统血管壁透壁性炎症的组织病理学证据是金标准，但由于其具有侵入性，通常在传统血管造影（CA）之后进行。PACNS的鉴别诊断范围很广，包括系统性血管炎、继发性血管炎、非炎性颅内血管病变、脱髓鞘疾病和肿瘤。这些其他疾病通常可以通过病史、检查、免疫检测、脑脊液分析和神经影像学来区分。CA可以检测大脑中到大动脉的血管炎变化，但特异性较低。血管壁成像技术的最新进展进一步有助于区分各种颅内血管病变与中枢神经系统血管炎。除非及时开始使用免疫抑制剂治疗，否则该病具有相当高的发病率和死亡率。以糖皮质激素和环磷酰胺进行诱导治疗，随后使用硫唑嘌呤、霉酚酸酯或甲氨蝶呤作为维持治疗，是治疗的基石。利妥昔单抗和抗肿瘤坏死因子α抑制剂（英夫利昔单抗和依那西普）等生物制剂可用于难治性病例。本文综述讨论了诊断方法、预后决定因素及治疗方法。
