系统性红斑狼疮中枢神经系统病变的诊断及治疗

目的 对系统性红斑狼疮（ＳＬＥ）患中枢神经系统病变（ＣＮＳ）的诊治经验进行总结,方法 对北京协和医院１７１例有ＣＮＳ的ＳＬＥ住院病例进行回顾性分析。结果（１）１７１例发病时ＳＬＥ病程为（２．２１±１．８７）年,１６３例（９５．３％）伴狼疮活动;（２）１５１例行脑脊液检查,异常１３８例（９１．４％）。其中蛋白,压力及白细胞增高分别为１１３．６９及５１例,糖降低仅６例;（３）对有ＣＮＳ的ＳＬＥ,头

Central nervous system involvement in systemic lupus erythematosus

OBJECTIVE: To investigate central nervous system (CNS) involvements in systemic lupus erythematosus (SLE) so as to enhance our knowledge in diagnosing and treating CNS involvement of SLE. METHODS: The clinical data of 171 in patients with CNS involvement of SLE in our hospital were retrospectively reviewed. RESULTS: The mean SLE disease duration at onset of CNS involvement was (2.21 +/- 1.87) years and in 163 (95.3%) it was associated with active disease. Cerebral spinal fluid abnormality was seen in 91.4% (138/151) of the patients with CNS involvement of SLE. Among them protein elevation was found in 113, pressure elevation in 69, white cell elevation in 51 and glucose reduction in 6. For the evaluation of CNS involvement of SLE, the sensitivity of cranial CT and MRI was 77.4% and 81.4% respectively (P > 0.50). The positive rate of antiribosomal P in patients with diffuse CNS involvement of SLE was significantly higher than that in patients without CNS involvement (P < 0.01). On the contrary, the positive rate of ACL in focal CNS involvement of SLE was significantly higher than that in diffuse type or in patients without CNS involvement (P < 0.01). The total mortality rate in 171 patients with CNS involvement of SLE was 18.7%. The Mortality rate in the period of 1993 to 1998 (4.0%, 3/75) was significantly lower than that of 1980 to 1992 (30.2%, 29/96), P < 0.01. 24 SLE patients with CNS involvement received intrathecal dexamethasone and methotrexate, 22 cases (91.7%) improved considerably. CONCLUSION: CNS involvement occurs in the early stage of SLE, most of the cases are associated with active disease. Cerebral spinal fluid analysis is the most essential test and cranial imaging serves as a supplementary approach, of which CT is preferred. ACL is associated with focal CNS involvement of SLE while antiribosomal P with diffuse CNS involvement of SLE, suggesting there might be different mechanisms in CNS involvement of SLE. Intrathecal therapy is an useful alternative for patients with CNS involvement of SLE refractory to conventional therapy.