神经精神狼疮的临床分型及其预后

Relationship between spectrum of neuropsychiatric lupus erythematosus and long term outcome

目的 了解神经精神狼疮 (NPLE)的临床分型 ,探讨其与狼疮疾病活动性、预后的关系。方法 对符合 1 999年美国风湿病学会 (ACR)神经精神狼疮定义标准的 70例SLE病人追踪 0 5年以上 ,了解NPLE的临床分型 ,采用系统性红斑狼疮疾病活动指数 (SLEDAI)和损伤指数对其进行评估 ,并分析死亡情况。结果 本组发现有 1 4种NPLE ,以癫痫样发作最常见 ,以下依次为无菌性脑膜炎、脱髓鞘综合征、急性意识障碍、精神病、狼疮样头痛、脑血管意外、脊髓损害、性格改变、焦虑、知觉异常、颅神经损害、运动障碍和吉兰—巴雷综合征。单种NPLE仅 2 4 % ,2种叠加 37% ,3种或以上39 %。NPLE多种叠加数与SLEDAI和损伤指数的积分均存在显著相关 ,与死亡情况相平行。结论 大部分NPLE存在多种叠加 ,不但提示疾病活动 ,而且意味着病情严重 ,预后较差 。

Objective To learn the spectrum of neuropsychiatric lupus erythematosus (NPLE) and the relationship between NPLE subgroups and disease activity or outcome.Methods Seventy patients who met the 1999 ACR definition critiria for NPLE were followed up more than 6 months.The correlations between NPLE subgroups and systemic lupus erythematosus disease activity index (SLEDAI) or damaging index were explored,and detail of death was analyzed.Results There were 14 subgroups of NPLE.The following frequency of NPLE were seizures and seizure disorders,aseptic meningitis,demyelinating syndrome,acute confusional state,psychosis,headache,cerebrovascular disease,myelopathy,mood disorders,anxiety disorder,cognitive dysfunction,cranial neuropathy,movement disorder (chorea) and Guillain Barré syndrome.The following morbilities were 24% in single NPLE group,37% in double,39% in three or more.The significant correlation between the number of NPLE and the scores of SLEDAI or damaging index were seen,whereas the number of NPLE was significantly possitive related to the mortality.Conclusion Most patients with NPLE have many kinds of manifestations.Multi manifestations not only suggest disease activity and disease severity,but higher morbility as well.