神经精神性狼疮30例临床分析

Clinical analysis of 30 cases of nervous systemic lupus erythematosus

目的：探讨系统性红斑狼疮（SLE）发生神经精神系统损害（NPLE）的临床特点和危险因素。方法回顾性分析160例 SLE 患者的临床资料，其中30例确诊 NPLE，分析 NPLE 与常见自身抗体、其他脏器损害的关系，并比较 SLE 疾病活动评分（SLEDAI）与 NPLE 的关联性。结果NPLE 患者血清中抗核抗体（ANA）、抗 Sm 抗体、抗 RNP 抗体、抗 SSA 抗体、抗 ds-DNA 抗体阳性率与无 NPLE 患者相比差异均无统计学意义（χ^2＝0．947、0．013、1．194、0．023、0．745，P ＝0．194、0．910、0．274、0．879、0．388）；肾脏损害、雷诺现象、间质性肺炎、肺血栓栓塞症、口腔溃疡、关节炎、颜面红斑、胸膜炎、心包炎、发热、肺动脉高压、光过敏、脱发在有无 NPLE 组间差异无统计学意义（χ^2＝0．419、1．383、0．721、0．201、1．368、1．194、0．055、0．946、0．262、2．503、0．628、2．898、0．075，P ＝0．517、0．324、0．396、1．000、0．242、0．274、0．815、0．331、0．609、0．114、0．428、0．089、0.785），而手足血管炎与 NPLE 的发生明显相关（χ^2＝3．996，P ＝0．046）；NPLE 组 SLEDAI 评分为（27．63±2.26）分，明显高于无 NPLE 组的（13．83±0．25）分（t ＝8．446，P ＝0．000）。结论NPLE 的发生与常见自身抗体阳性无相关性，与其他脏器受累无关，而手足血管炎、SLEDAI 评分＞15分是 NPLE 发生的危险因素。部分病例可以脑病为首发表现。及早诊断、甲泼尼龙冲击联合免疫抑制剂治疗可以有效提高疾病缓解率，减少死亡，改善预后。

Objective To invetigate clinical characteristics,pathogenesis and its risk factors of neuropsychi-atric lupus erythematosus（NPLE）.Methods 160 cases of SLE patients,including 30 cases of diagnosed NPLE,were analyzed retrospectively.The relationship between the appearance of NPLE and common autoantibodies,other organ involvement and SLE disease activity score（SLEDAI）were analyzed.Results Such antibody positive rates of anti nuclear antibody（ANA）,anti Sm antibody,anti -RNP antibody,anti SSA antibody,anti ds -DNA antibody had no significant difference between NPLE and non NPLE,the value of χ2 was 0.947,0.013,1.194,0.023,0.745 respectively,the value of P was 0.194,0.910,0.274,0.879,0.388 respectively.Renal involvement,raynaud phenomenon,interstitial pneumonia,pulmonary thromboembolism,oral ulcer,arthritis,facial erythema,pleuritis,pericarditis,fever,pulmonary hypertension,photo -allergy and alopecia in the two groups had no difference,the value of χ2 was respectively 0.419,1.383,0.721,0.201,1.368,1.194,0.055,0.946,0.262,2.503,0.628,2.898 and 0.075,the value of P was 0.517,0.324,0.396,1.000,0.242,0.274,0.815,0.331,0.609,0.114,0.428,0.089 and 0.785 respectively.But the occurrence of hand and foot vasculitis in NPLE was significantly higher（χ2 =3.996,P =0.046）.SLEDAI of NPLE was higher than non NPLE（t =8.446,P =0.000）.Conclusion There was no correlation with the occurrence of NPLE and common autoantibodies,other organ involvements.Hand and foot vasculitis and higher SLEDAI（more than 15 points）were the risk factors of NPLE.Encephalopathy may be the initial manifestation to some of SLE cases. Early diagnosis and methylprednisolone pulse treatment combined with immunosuppressive therapy can effectively improve the remission of the disease,reduce mortality and improve prognosis.