Bortoluzzi A, Padovan M, Farina I, Galuppi E, De Leonardis F, Govoni M
Unità Operativa Complessa e Sezione di Reumatologia, Dipartimento di Medicina Clinica e Sperimentale, Università di Ferrara e Azienda Ospedaliero-Universitaria S. Anna, Ferrara, Italy.
Reumatismo. 2012 Dec 20;64(6):350-9. doi: 10.4081/reumatismo.2012.350.
The management of neuropsychiatric systemic lupus erythematosus (NPSLE) still remains empirical and based on clinical experience due to the lack of randomized controlled trials.
To report the experience accumulated in a single tertiary referral centre about treatment of severe cases of NPSLE patients and to discuss therapeutic strategies on the background of EULAR recommendations.
Retrospective analysis of all consecutive cases of severe NPSLE treated in our centre since 1990 to 2010, satisfying the 1999 ACR criteria.
Among 633 SLE patients who consecutively attended our centre, 231 (36%) displayed at least one neuropsychiatric (NP) manifestation for a total of 408 events attributable to SLE. Thirty-one patients (4.8%), 27 females and 4 males, experienced 35 major NP events requiring immunosuppressive therapy (including 3 relapses and 1 new event). An aggressive immunosuppressive strategy was applied to those patients with an immune mediated inflammatory NP event and to those patients with an increased disease activity as judged by ECLAM and SLEDAI scores. Overall at the end of the therapy 74% of the patients reached clinical remission or significant improvement of their symptoms measured by mean SLEDAI (from 10.09 ± 1.09 to 2.04 ± 0.52, P<0.0001) and ECLAM (from 4 ± 0.34 to 1.38 ± 0.37, P<0.001) scores.
The prevalence of NP involvement, described in our case series, is similar to those reported in literature as well as the treatment strategies applied. Nowadays, it is not possible to establish a standardized approach for each single NPSLE manifestation, and different therapeutic strategies must be tailored taking into account the most probable pathogenic mechanism involved, the general disease activity background, the co-morbidities, the type and the stage of the systemic involvement.
由于缺乏随机对照试验，神经精神性系统性红斑狼疮（NPSLE）的治疗仍基于临床经验。
报告在一家三级转诊中心积累的关于治疗重症NPSLE患者的经验，并在欧洲抗风湿病联盟（EULAR）建议的背景下讨论治疗策略。
回顾性分析自1990年至2010年在我们中心治疗的所有符合1999年美国风湿病学会（ACR）标准的重症NPSLE连续病例。
在连续就诊于我们中心的633例SLE患者中，231例（36%）出现至少一种神经精神（NP）表现，共计408次事件归因于SLE。31例患者（4.8%），27例女性和4例男性，经历了35次需要免疫抑制治疗的严重NP事件（包括3次复发和1次新事件）。对于那些有免疫介导的炎症性NP事件以及根据欧洲狼疮活动度量表（ECLAM）和系统性红斑狼疮疾病活动指数（SLEDAI）评分判断疾病活动度增加的患者，采用了积极的免疫抑制策略。总体而言，治疗结束时，74%的患者达到临床缓解或症状有显著改善，平均SLEDAI评分（从10.09±1.09降至2.04±0.52，P<0.0001）和ECLAM评分（从4±0.34降至1.38±0.37，P<0.001）。
我们病例系列中描述的NP受累患病率与文献报道的以及所应用的治疗策略相似。如今，不可能为每种单一的NPSLE表现建立标准化方法，必须根据最可能涉及的致病机制、一般疾病活动背景、合并症、系统性受累的类型和阶段来制定不同的治疗策略。
