甲氨蝶呤是一种常用于治疗炎症性疾病，如类风湿关节炎的免疫抑制剂。然而，尽管非常罕见，它可能对中枢神经系统（CNS）产生严重不良反应，如甲氨蝶呤相关淋巴增生疾病（MTX-LPD）。目前对于中枢神经系统MTX-LPD患者的自然病程、治疗选择和临床结果的文献报道非常有限。我们根据PRISMA指南进行了系统文献综述，并提供了一个中枢神经系统MTX-LPD的案例说明。文献综述显示已有12例CNS MTX-LPD的发表病例，再加上本案例一共包括13例。使用甲氨蝶呤最常见的适应症是类风湿关节炎。对于淋巴增生疾病的最常见治疗方法是停用甲氨蝶呤（12例，92.3%）、辅助化疗（2例，15.4%）、全肿瘤切除（3例，23.1%）或激素治疗（1例，7.7%）。治疗通常导致神经症状的改善（9例，69.2%），病变的消退（3例，23.1%）并且没有复发（6例，46.2%）。四例（4例，30.8%）报道了死亡病例，平均发病时间为11个月。对于正在使用甲氨蝶呤并出现神经症状的患者，应将中枢神经系统MTX-LPD包括在鉴别诊断中，因为立即停用甲氨蝶呤显示了良好的预后。© 2023 Elsevier Inc. 保留所有权利。

Methotrexate is an immunosuppressant commonly used to treat inflammatory conditions, such as rheumatoid arthritis. However, albeit exceedingly rare, it can have serious adverse effects within the central nervous system (CNS), such as methotrexate-associated lymphoproliferative disorder (MTX-LPD). Literature describing the natural history, treatment options, and clinical outcomes of patients with central nervous system MTX-LPD remains sparse.We present a systematic literature review following PRISMA guidelines and a case illustration of CNS MTX-LPD.A systematic review of the literature revealed 12 published cases of CNS MTX-LPD, plus the case presented herein, for a total of 13 included cases. The most common indication for MTX was rheumatoid arthritis. The most common treatment for the LPD was MTX cessation (12, 92.3%), adjunct chemotherapy (2, 15.4%), total tumor resection (3, 23.1%), or steroid therapy (1, 7.7%). Treatment usually led to improvement of neurological symptoms (9, 69.2%) along with regression of the lesions (3, 23.1%) with no recurrence (6, 46.2%). Death was reported in four cases (4, 30.8%) with a mean time from onset of 11 months.CNS MTX-LPD should be considered in the differential diagnosis for patients who are taking MTX presenting with neurologic symptoms, as immediate withdrawal of MTX has demonstrated good prognosis.Copyright © 2023 Elsevier Inc. All rights reserved.