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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 ,病死率较高
Objective To investigate the clinical classification of neuroleptic lupus (NPLE) and explore its relationship with the activity and prognosis of lupus disease. Methods Totally 70 cases of SLE patients meeting the definition criteria of neuroleptic lupus of American College of Rheumatology (ACR) in 1999 were followed for more than 5 years to understand the clinical classification of NPLE. The systemic lupus erythematosus disease activity index (SLEDAI) and injury index Evaluate it and analyze the death. Results The group found 14 kinds of NPLE, the most common epileptic seizures, followed by aseptic meningitis, demyelinating syndrome, acute disturbance of consciousness, mental illness, lupus-like headache, cerebrovascular accident, spinal cord injury, personality Change, anxiety, abnormality of consciousness, cranial nerve damage, dyskinesia and Guillain-Barre syndrome. A single NPLE was only 24%, two overlaid 37%, three or more 39%. There were significant correlations between the NPLE superposition numbers and the integrals of SLEDAI and injury index, parallel with the death. Conclusions Most of the NPLEs have multiple superpositions, which not only indicate disease activity, but also indicate serious illness, poor prognosis and high mortality