神经精神性SLE临床特征分析

Clinical features of neuropsychiatric systemic lupus erythematosus

目的探讨神经精神性SLE(NPSLE)患者发病的危险因素、临床表现和治疗。方法回顾分析42例NPSLE患者(NPSLE组)的临床资料,并根据美国风湿病协会(ACR)制订的NPSLE命名和19种临床类型分型。设同期住院的187例无神经精神症状的SLE患者为对照组(非NPSLE组),比较两组患者的临床表现、实验室指标及狼疮疾病活动性指数(SLEDAI)。结果NPSLE组患者50%发作前有明确的诱因,42例患者共出现12种症状类型,73.8%患者有两种及其以上表现,以癫痫(27.0%)、脑血管病变(20.6%)和急性精神错乱(14.3%)最常见。NPSLE组发热、红细胞沉降率升高、血小板减少的发生率、SLEDAI分值以及死亡率分别高于非NPSLE组。差异有统计学意义(P<0.05)。盘状红斑、关节痛的发生率以及正规使用糖皮质激素或合用免疫抑制剂的患者比例分别低于非NPSLE组.差异有统计学意义(P<0.05)。NPSLE患者死亡原因有疾病本身、并发感染、重要脏器功能衰竭。结论NPSLE患者病情严重.疾病活动性高,预后差。正规治疗和预防并发症能避免诱发NPSLE,同时能改善其预后。

Objective To investigate the risk factors, clinical manifestations and treatment ofneuropsychiatric systemic lupus erythematosus （ NPSLE ）. Methods Clinical data, including clinical manifestations, laboratory examination results and SLE disease activity index （ SLEDAI ） score, were retrospectively analyzed for 42 patients with NPSLE and 187 patients with non-NPSLE as controls. Neuropsychiatric manifestations were classified according to the American College ofRheumatology （ ACR ） nomenclature and case definition for NPSLE. Results Of all NPSLE patients, 50% had definite precipitating cause before neuropsychiatric symptoms occurred. A total of 12 types of neuropsychiatric manifestation were observed, and 73.8% patients presented two or more clinical types. The most common symptoms involved epilepsy （ 27.0% ）, cerebrovascular disease （ 20.6% ） and acute confusional states （ 14.3% ）. The incidence of fever, elevated ESR and thrombocytopenia, as well as the mean SLEDAI score and mortality rate were higher in NPSLE group than in non-NPSLE group （ all P 〈 0.05 ）. However, the incidence of discoid erythema and arthralgia, as well as the percentage of the patients receiving regular treatment with steriods or combination therapy with immunosuppressants were significantly lower in NPSLE group than in non-NPSLE group （ all P 〈 0.05 ）. The causes of death included NPSLE itself and complicated infection and organ failures. Conclusions The patients with NPSLE usually have a more severe condition, higher disease activity and worse prognosis than those with non-NPSLE. Regular treatment and prevention of complications with systemic steriods and immunosuppressants might prevent the occurrence of NPSLE and improve the prognosis of SLE.