Fujieda Yuichiro
Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Immunol Med. 2020 Dec;43(4):135-141. doi: 10.1080/25785826.2020.1770947. Epub 2020 May 27.
Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterised by diverse organ damages resulting from various autoantibodies, such as antinuclear or anti-DNA antibodies. Neuropsychiatric lupus (NPSLE) refers to the neurological and psychiatric disorders complicated with SLE and can be challenging for physicians to manage. NPSLE has a broad spectrum and high heterogeneity of clinical phenotypes, including headaches, psychiatric symptoms and peripheral neuropathy. Additionally, various immune effectors have been reported to contribute to the pathogenesis, including cytokines, cell-mediated inflammation and brain-reactive autoantibodies. In some patients with SLE, neuropsychiatric symptoms develop for the first time after the initiation of the steroid treatment, hindering the differentiation from steroid psychosis. The administration of high doses of steroids in patients with SLE is believed to trigger psychiatric symptoms. No clear evidence has yet been found regarding the treatment of NPSLE. Therefore, NPSLE-specific markers need to be developed, and treatment guidelines should be established. This article provides an overview of NPSLE as well as its pathogenesis and treatment.
系统性红斑狼疮（SLE）是一种全身性自身免疫性疾病，其特征是由各种自身抗体（如抗核抗体或抗DNA抗体）导致的多种器官损害。神经精神性狼疮（NPSLE）是指并发于SLE的神经和精神障碍，对医生的治疗而言可能具有挑战性。NPSLE具有广泛的临床表型谱和高度异质性，包括头痛、精神症状和周围神经病变。此外，据报道各种免疫效应器参与了其发病机制，包括细胞因子、细胞介导的炎症和脑反应性自身抗体。在一些SLE患者中，神经精神症状在开始使用类固醇治疗后首次出现，这阻碍了与类固醇精神病的鉴别。SLE患者使用高剂量类固醇被认为会引发精神症状。关于NPSLE的治疗尚未找到明确的证据。因此，需要开发NPSLE特异性标志物，并建立治疗指南。本文概述了NPSLE及其发病机制和治疗方法。
