Primary central nervous system lymphoma (PCNSL) sometimes occurs in immune-compromised hosts or patients with autoimmune diseases. Some cohort studies have previously reported an increased risk of non-Hodgkin’s lymphoma in systemic lupus erythematosus (SLE), while some cases of PCNSL in patients with SLE were reported. We present the case of PCNSL which developed in a patient with the active phase of neuropsychiatric SLE (NPSLE). Furthermore, we reviewed published English articles to confirm the characteristics of PCNSL related to SLE. To our knowledge, this is the first report of PCNSL occurring in NPSLE. Histology demonstrated B-cell lymphoma with a positive Epstein–Barr virus-encoded RNA. This patient recovered following surgical resection of the lymphoma, whole brain radiation therapy, intravenous infusion of rituximab (RTX), and administration of belimumab after RTX. Given the series of reviews, our report suggests that the persistence of damage in the central nervous system (CNS) and long-term exposure to immunosuppressants may impact oncogenic immune responses within the CNS, leading to PCNSL development.

原发性中枢神经系统淋巴瘤 (PCNSL) 有时发生在免疫受损的宿主或患有自身免疫性疾病的患者中。一些队列研究之前曾报道过系统性红斑狼疮 (SLE) 患非霍奇金淋巴瘤的风险增加，而一些 SLE 患者患 PCNSL 的病例也有报道。我们介绍了在神经精神系统性红斑狼疮 (NPSLE) 活动期患者中发生的 PCNSL 病例。此外，我们回顾了已发表的英文文章，以确认 PCNSL 与 SLE 相关的特征。据我们所知，这是 NPSLE 中发生 PCNSL 的第一份报告。组织学证明 B 细胞淋巴瘤具有阳性 Epstein-Barr 病毒编码的 RNA。该患者在手术切除淋巴瘤、全脑放射治疗、静脉输注利妥昔单抗 (RTX)、RTX 后给予贝利木单抗。鉴于一系列审查，我们的报告表明，中枢神经系统 (CNS) 损伤的持续存在和长期暴露于免疫抑制剂可能会影响 CNS 内的致癌免疫反应，导致 PCNSL 的发展。