神经精神狼疮患者240例的临床特点、治疗及预后分析

Neuropsychiatric manifestations in systemic lupus erythematosus and the treatment of intrathecal methotrexate plus dexamethasone

目的对神经精神狼疮（NPSLE）的临床表现、治疗及预后进行总结。方法对240例NPSLE患者进行回顾性分析，根据1999年美国风湿病学会（ACR）对神经精神狼疮命名和定义的分类标准对NPSLE患者分型，并分析鞘内注射甲氨蝶呤（MTX）及地塞米松（DXM）治疗NPSLE的疗效。结果240例患者共表现15种神经精神症状，最常见为狼疮性头痛及癫痫样发作，64．2％（154／240）患者同时存在两种及以上神经精神症状。109例患者接受鞘内注射治疗，接受鞘内注射患者组的治疗后狼疮疾病活动指数（SLEDAI，6±5）、平均住院日（39d±22d）及住院病死率（6．6％）均明显低于对照组（10±6，46d±26d，14．8％）（P〈0．05～0．01），鞘内注射治疗的副作用发生率为11．0％（12／109）。130例随诊患者有23例复发，其中7例给予鞘内注射治疗均获得再次缓解。结论神经精神狼疮的临床表现多样，常见为狼疮性头痛及癫痴样发作。鞘内注射MTX及DXM治疗能明显改善NPSLE的预后。

Objective To study the neuropsychiatric （NP） manifestations of systemic lupus erythematosus （SLE） and evaluate treatment with intrathecal （IT） methotrexate（MTX） and dexamethasone （DXM）. Methods 240 patients with NP syndromes of SLE （NPSLE） from 1990 to 2004 were retrospectively reviewed and IT injection group and non-IT injection group were compared. The side effects of IT injection were also discussed. 130 patients were followed up after discharge. Results Fifteen of the 19 ACR NP syndromes were identified. 86（35.8% ） patients presented one NP syndrome and 154 （64. 2% ） presented with more than one. The most frequent manifestations were headache and seizure disorder. 109 patients received IT injections. After IT injection, the CSF index had significantly improved. The mean SLEDAI score, mean duration of hospitalization and mortality rate of patients with IT injection were lower than those patients without IT injection （ P 〈 0. 05-0. 001 ）. The side effects of IT injection were found in 11.0% patients. 23 of 130 patients had recurrent NP events during follow up. Conclusion There was heterogeneity of NPSLE in our study group. The most common NP features were headache and seizure. IT injection of MTX and DXM is an effective and safe alternative to traditional treatment of NPSLE.