Department of Rheumatology, Leiden University Medical Centre (LUMC), Leiden, The Netherlands
Department of Rheumatology, Leiden University Medical Centre (LUMC), Leiden, The Netherlands.
Lupus Sci Med. 2023 Feb;10(1). doi: 10.1136/lupus-2022-000850.
The short-term and long-term outcome of inflammatory neuropsychiatric SLE (NPSLE) with immunosuppressive treatment is largely unknown. We used clinical data from our tertiary referral centre for NPSLE to investigate the type of inflammatory NPSLE manifestations, type of immunosuppressive treatment prescribed for these manifestations and clinical outcomes.
All patients with SLE visiting the Leiden University Medical Centre NPSLE clinic between 2007 and 2021 receiving immunosuppressive therapy for neuropsychiatric symptoms were included. Clinical, immunological and radiological information was collected in as standardised way during a 1-day multidisciplinary assessment. In a multidisciplinary consensus meeting, the presence of NPSLE and the type of NPSLE manifestations and treatment were determined. For this study, short-term (0-6 months) and long-term outcomes (7-24 months) of the NP symptoms were assessed by two independent readers and scored on a 7-point Likert scale, ranging from death to resolved.
In total, 95 out of 398 (24%) patients visiting the NPSLE clinic between 2007 and 2021 received any form of immunosuppressive treatment for 101 separate NPSLE events. The most common NP manifestation was cognitive dysfunction (50%) as identified by formal cognitive assessment, often present in combination with other NPSLE manifestations. Treatment modalities were induction (24%), induction and maintenance (73%) and other therapy (3%). The treatments mostly consisted of (combinations of) prednisone (97%), methylprednisolone (53%), azathioprine (generally 2 mg/kg daily) (49%) and cyclophosphamide (generally induction 750 mg/m every 4 weeks for 24 weeks or 500mg biweekly for 12 weeks) (42%). Short-term outcome showed improvement on the Likert scale in 73% (improved: 22%, much improved: 29%, resolved: 22%), no change in 21% and worsening in 6% of patients. Long-term outcome was available for 78 out of 101 events and showed improvement in 70% (improved: 14%, much improved: 28%, resolved: 28%), no change in 17%, worsening in 10% and death in 3% of patients (none directly NPSLE-related).
The outcome of inflammatory NPSLE after immunosuppressive treatment is generally good, with improvement of neuropsychiatric symptoms occuring in approximately 70% of events.
炎性神经精神性狼疮（NPSLE）患者接受免疫抑制治疗后的短期和长期结局在很大程度上尚不清楚。我们使用来自我们的三级转诊中心的 NPSLE 临床数据来研究炎性 NPSLE 表现的类型、为这些表现开具的免疫抑制治疗类型以及临床结局。
纳入 2007 年至 2021 年间在莱顿大学医学中心 NPSLE 诊所就诊并因神经精神症状接受免疫抑制治疗的所有 SLE 患者。在为期 1 天的多学科评估中，以标准化方式收集临床、免疫和影像学信息。在多学科共识会议上，确定了 NPSLE 的存在以及 NPSLE 表现和治疗的类型。对于这项研究，两名独立的读者评估了 NP 症状的短期（0-6 个月）和长期（7-24 个月）结局，并使用 7 分李克特量表进行评分，范围从死亡到缓解。
在 2007 年至 2021 年间，总共 398 名就诊于 NPSLE 诊所的患者中有 95 名（24%）因 101 次单独的 NPSLE 事件接受了任何形式的免疫抑制治疗。最常见的 NP 表现是认知功能障碍（50%），通过正式的认知评估确定，通常与其他 NPSLE 表现同时存在。治疗方式为诱导治疗（24%）、诱导和维持治疗（73%）和其他治疗（3%）。治疗大多包括（组合）泼尼松（97%）、甲泼尼龙（53%）、阿扎胞苷（通常为 2mg/kg/天）（49%）和环磷酰胺（通常为诱导治疗，每 4 周 750mg/m2 共 24 周，或每 2 周 500mg 共 12 周）（42%）。短期结局的 Likert 量表评分显示，73%的患者有所改善（改善：22%，明显改善：29%，缓解：22%），21%的患者无变化，6%的患者恶化。78 例 101 例事件中有 78 例可获得长期结局，70%（改善：14%，明显改善：28%，缓解：28%）的患者有所改善，17%的患者无变化，10%的患者恶化，3%的患者死亡（均与 NPSLE 无关）。
炎性 NPSLE 患者接受免疫抑制治疗后的结局总体良好，大约 70%的事件中神经精神症状得到改善。
