CAJ | 학술논문

目的：了解系统性红斑狼疮（ SLE）伴中枢神经系统（ CNS）损害的临床特点。方法选取2002年1月—2014年12月首都医科大学附属北京天坛医院收治的SLE伴CNS损害患者49例，分析CNS损害出现时间、首诊科室、临床表现、脑脊液检查、颅脑CT和/或MRI检查、外周血检查、治疗及转归。结果 CNS损害出现时间≤1年的患者17例（34.7%），>1～3年的患者19例（38.8%），>3～5年的患者7例（14.3%），>5年的患者6例（12.2%）。以肾内科为首诊科室14例（28.6%），皮肤科12例（24.5%），血液科8例（16.3%），呼吸内科7例（14.3%），神经内科6例（12.2%），消化内科2例（4.1%）。SLE 伴 CNS 损害的临床表现主要为癫痫，发生率为26.5%（13/49），精神障碍和脑梗死发生率均为14.3%（7/49）。49例患者中34例完成脑脊液检查，其中脑脊液蛋白升高7例（14.3%），脑脊液免疫球蛋白G（CSF-IgG）指数异常4例（8.2%）。颅脑CT和/或MRI检查发现新发脑梗死10例（20.4%），脑白质脱髓鞘改变9例（18.4%），微出血6例（12.2%）。外周血检查抗核抗体（ANA）阳性21例（42.9%），抗ds-DNA抗体阳性19例（38.8%）。34例患者应用甲泼尼龙联合免疫抑制剂硫唑嘌呤等、神经营养药、抗凝剂和改善脑循环药物治疗，随访3年好转18例、无改变9例、加重7例；15例患者应用神经营养药、改善脑循环药物等对症支持治疗，随访3年病情加重7例、无改变6例、死亡2例。结论 SLE起病到CNS损害出现时间较短，首诊科室以肾内科和皮肤科多见，CNS损害的临床表现主要为癫痫、精神障碍和脑梗死，实验室检查可发现脑脊液及颅脑CT和/或MRI异常，应早期诊断及早期治疗。
목적：료해계통성홍반랑창（ SLE）반중추신경계통（ CNS）손해적림상특점。방법선취2002년1월—2014년12월수도의과대학부속북경천단의원수치적SLE반CNS손해환자49례，분석CNS손해출현시간、수진과실、림상표현、뇌척액검사、로뇌CT화/혹MRI검사、외주혈검사、치료급전귀。결과 CNS손해출현시간≤1년적환자17례（34.7%），>1～3년적환자19례（38.8%），>3～5년적환자7례（14.3%），>5년적환자6례（12.2%）。이신내과위수진과실14례（28.6%），피부과12례（24.5%），혈액과8례（16.3%），호흡내과7례（14.3%），신경내과6례（12.2%），소화내과2례（4.1%）。SLE 반 CNS 손해적림상표현주요위전간，발생솔위26.5%（13/49），정신장애화뇌경사발생솔균위14.3%（7/49）。49례환자중34례완성뇌척액검사，기중뇌척액단백승고7례（14.3%），뇌척액면역구단백G（CSF-IgG）지수이상4례（8.2%）。로뇌CT화/혹MRI검사발현신발뇌경사10례（20.4%），뇌백질탈수초개변9례（18.4%），미출혈6례（12.2%）。외주혈검사항핵항체（ANA）양성21례（42.9%），항ds-DNA항체양성19례（38.8%）。34례환자응용갑발니룡연합면역억제제류서표령등、신경영양약、항응제화개선뇌순배약물치료，수방3년호전18례、무개변9례、가중7례；15례환자응용신경영양약、개선뇌순배약물등대증지지치료，수방3년병정가중7례、무개변6례、사망2례。결론 SLE기병도CNS손해출현시간교단，수진과실이신내과화피부과다견，CNS손해적림상표현주요위전간、정신장애화뇌경사，실험실검사가발현뇌척액급로뇌CT화/혹MRI이상，응조기진단급조기치료。
Objective To investigate the clinical features of systemic lupus erythematosus( SLE)complicated with central nervous system ( CNS ) damage. Methods Enrolled 49 patients with SLE complicated with CNS damage who were admitted into Beijing Tiantan Hospital,Capital Medical University from January 2002 to December 2014. Analyzed the time when CNS damage occurred,the department for the first diagnosis,clinical manifestations,cerebrospinal fluid examination,cerebral CT and/or MRI,peripheral blood( PB),treatment and outcome. Results The numbers of patients with the time range from SLE onset to CNS damage ≤1 year,>1 -3 years,>3 -5 years and >5 years were 17 ( 34. 7%),19 ( 38. 8%),7 (14. 3%)and 6(12. 2%)respectively. The numbers of patients with nephrology department,dermatological department, hematology department, department of respiration medicine, neurology department and gastroenterology department as the department for the first diagnosis were 14 ( 28. 6%), 12 ( 24. 5%), 8 ( 16. 3%), 7 ( 14. 3%), 6 ( 12. 2%) and 2 (4. 1%)respectively. The major clinical manifestations of SLE complicated with CNS damage were epilepsy with a prevalence of 26. 5%(13/49),mental disorder with a prevalence of 14. 3%(7/49) and cerebral infarction with a prevalence of 14. 3%(7/49). There were 34 patients who finished the cerebrospinal fluid examination,among which 7(14. 3%)had elevated protein and 4 ( 8. 2%) had abnormal CSF - IgG. The cerebral CT and/or MRI showed that 10 ( 20. 4%) had newly found cerebral infarction, 9 ( 18. 4%) had changes in demyelination in the cerebral white matter, 6 ( 12. 2%) had micro bleeding. The PB examination showed that 21 ( 42. 9%) had positive ANA and 19 ( 38. 8%) had positive anti - ds DNA antibody. There were 34 patients who were administrated with methylprednisolone combined with azathioprine and other immunosuppressors,neurotrophy medicine,anticoagulant and agents improving cerebral circulation;after a 3-year follow-up, 18 patients had their condition improved,9 patients had no improvement,and 7 patients exacerbated. There were 15 patients who were administrated with symptomatic and supportive treatment,such as trophic nerve and microcirculation improvement;after a 3-year follow-up,7 patients exacerbated,6 patients had no improvement,and 2 patients died. Conclusion The time range from SLE onset to CNS damage is short,the departments for the first diagnosis are mostly neurology department and dermatological department,and the clinical manifestations of CNS damage are mainly epilepsy,mental disorder and cerebral infarction. Through laboratory examination, abnormality of cerebrospinal fluid and cerebral CT and/or MRI could be found, and early - stage treatment and early treatment should be undertaken.