Adjunctive plasma exchanges to treat neuropsychiatric lupus:: a retrospective study on 10 patients

Adjunctive plasma exchanges to treat neuropsychiatric lupus:: a retrospective study on 10 patients
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DOI:
10.1177/0961203307081840
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发表时间:
2007-01-01
期刊:
影响因子:
2.6
通讯作者:
Guillevin, L.
Guillevin, L.
中科院分区:
医学4区
文献类型:
--
作者:
Bartolucci, P.;Brechignac, S.;Guillevin, L.

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系统性红斑狼疮(NPSLE)的神经精神表现是狼疮活动导致发病率和死亡率的主要原因之一。传统的NPSLE治疗结合CS和免疫抑制剂,但一些症状没有反应。我们回顾性评估了1989年至2002年间10例患者(平均年龄30岁)的13例NPSLE耀斑合并血浆置换(PE)的治疗效果。NP表现是7例患者的首发SLE症状,平均有3.2个NP表现/发作。所有患者均接受CS和环磷酰胺脉冲治疗。平均进行15次PE/flare。所有患者在术后平均3周(中位数:2.5周;范围:1.5-8周)内得到改善。7/13的耀斑在平均7周(中位数:4;范围:2-22)内得到完全缓解。其余六个耀斑部分缓解,其特征是在三个期间出现新的NP表现,而其他耀斑控制不足。所有患者的其他SLE表现均有所退化,欧洲共识狼疮活动测量平均分从预处理6.9降至1.2。CS、环磷酰胺和PE联合治疗方案对严重NPSLE有效,毒性可接受。
Neuropsychiatric manifestations of systemic lupus erythematosus (NPSLE) are among the main causes of morbidity and mortality attributed to lupus activity. Conventional NPSLE treatment combines CS and immunosuppressants, but some symptoms do not respond. We retrospectively evaluated the adjunction of plasma exchanges (PE) to treat 13 NPSLE flares occurring in 10 patients (mean age, 30 years) between 1989 and 2002. NP manifestations were the first SLE symptoms for seven patients, with a mean of 3.2 NP manifestations/flare. All patients received CS and cyclophosphamide pulses. A mean of 15 PE/flare were performed. All patients improved within a mean of 3 (median: 2.5; range: 1.5-8) weeks thereafter. Complete remissions of 7/13 flares were obtained within a mean of 7 (median: 4; range: 2-22) weeks. Partial remissions were achieved for the remaining six flares, characterized by new NP manifestations during three and insufficient control of the others. Other SLE manifestations regressed for all patients with the mean European consensus lupus activity measurement score declining from pretreatment 6.9 to 1.2. A regimen combining CS, cyclophosphamide and PE is effective against severe NPSLE, with acceptable toxicity.