Division of Rheumatology, Medical University of Vienna, Spitalgasse 23, 1090 Vienna, Austria.
Curr Pharm Des. 2022;28(11):881-891. doi: 10.2174/1381612828666220512102824.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease, which affects a wide range of organs with variable clinical features. Involvement of the nervous system is a challenging and multifaceted manifestation of the disease, presenting with a broad range of symptoms. Neuropsychiatric lupus (NPSLE) encompasses seven syndromes of the peripheral and 12 of the central nervous system, associated with a high disease burden. Despite advances in the management of SLE, NP manifestations still pose a challenge to clinicians. First, diagnosis and attribution of SLE are difficult due to the lack of specific biomarkers or imaging modalities. Second, therapeutic options are limited, and evidence is mainly based on case reports and expert consensus, as clinical trials are sparse. Moreover, no validated outcome measure on disease activity exists. Current recommendations for treatment include supportive as well as immunosuppressive medication, depending on the type and severity of manifestations. As NPSLE manifestations are increasingly recognized, a broader spectrum of therapeutic options can be expected.
系统性红斑狼疮（SLE）是一种自身免疫性疾病，可影响多个器官，具有多种不同的临床表现。神经系统受累是疾病的一个具有挑战性且多方面的表现，可出现广泛的症状。神经精神狼疮（NPSLE）包括七种周围神经系统综合征和十二种中枢神经系统综合征，与高疾病负担相关。尽管 SLE 的治疗取得了进展，但 NP 表现仍然对临床医生构成挑战。首先，由于缺乏特异性生物标志物或影像学手段，SLE 的诊断和归因具有一定难度。其次，治疗选择有限，证据主要基于病例报告和专家共识，因为临床试验很少。此外，目前尚无针对疾病活动的验证后测量方法。目前的治疗建议包括支持治疗和免疫抑制治疗，具体取决于表现的类型和严重程度。随着 NPSLE 表现的日益被认识，预计会有更广泛的治疗选择。
