Department of Neurology, Focus Program Translational Neuroscience (FTN), Rhine Main Neuroscience Network (rmn(2)), University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany; Department of Neurology, Philipps-University Marburg, Baldingerstr, 35043 Marburg, Germany.
Department of Neurology, Focus Program Translational Neuroscience (FTN), Rhine Main Neuroscience Network (rmn(2)), University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany.
Autoimmun Rev. 2024 Apr;23(4):103528. doi: 10.1016/j.autrev.2024.103528. Epub 2024 Mar 16.
The occurrence of cerebral vasculitis in individuals with neurosarcoidosis (NS) is considered to be rare. Although the number of relevant publications has increased in recent years, evidence is mostly limited to case reports. To obtain a better understanding of this rare and severe manifestation of disease, we carried out a scoping review on cerebral vasculitis in patients diagnosed with NS. The results of the review indicate that the diagnosis of cerebral vasculitis in patients with NS is made especially in patients with systemic sarcoidosis. However, recurrent strokes in patients with NS remains the main indicator of cerebral vasculitis. A tissue biopsy is considered the gold standard to confirm the diagnosis despite occasional false-negative results. Glucocorticoids and steroid-sparing agents are the most successful current treatments. Favorable outcomes were observed with strategies targeting TNFα and B cells. The goal of this review is to summarize the current literature and treatment options for cerebral vasculitis in patients with NS.
神经结节病患者出现脑血管炎被认为较为罕见。尽管近年来相关出版物的数量有所增加,但证据大多仅限于病例报告。为了更好地了解这种罕见且严重的疾病表现,我们对诊断为神经结节病患者的脑血管炎进行了范围性综述。综述结果表明,尤其在系统性结节病患者中诊断出神经结节病患者的脑血管炎。然而,神经结节病患者反复发生中风仍然是脑血管炎的主要指标。尽管偶尔会出现假阴性结果,但组织活检被认为是确诊的金标准。糖皮质激素和类固醇节约剂是目前最成功的治疗方法。针对 TNFα 和 B 细胞的治疗策略观察到了良好的效果。本综述的目的是总结神经结节病患者脑血管炎的当前文献和治疗选择。