General Neurology Division, Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Brazil; Hospital Israelita Albert Einstein, São Paulo, Brazil.
Rheumatology Division, Universidade Federal de São Paulo, São Paulo, Brazil.
Autoimmun Rev. 2017 Feb;16(2):123-131. doi: 10.1016/j.autrev.2016.12.001. Epub 2017 Jan 11.
Primary central nervous system vasculitis (PCNSV) is a challenging diagnosis due to broad clinical manifestations and variable specificity and sensitivity of laboratory and imaging diagnostic tools. Differential diagnosis includes reversible cerebral vasoconstriction syndrome (RCVS), secondary vasculitis of the CNS and other noninflammatory vasculopathies. Brain biopsy is essential for definitive diagnosis and to exclude mimickers. Recent data show that data large-vessel PCNSV present worse prognosis when compared to small-vessel PCNSV. Herein we review diagnosis and management of PCNSV, secondary vasculitis of CNS and RCVS.
原发性中枢神经系统血管炎（PCNSV）是一种具有挑战性的诊断，因为其临床表现广泛，且实验室和影像学诊断工具的特异性和敏感性各不相同。鉴别诊断包括可逆性脑血管收缩综合征（RCVS）、中枢神经系统继发性血管炎和其他非炎症性血管病。脑活检对于明确诊断和排除类似疾病至关重要。最近的数据表明，与小血管 PCNSV 相比，大血管 PCNSV 的预后更差。在此，我们回顾了 PCNSV、中枢神经系统继发性血管炎和 RCVS 的诊断和治疗。
