CAJ | 학술논문

目的：总结系统性红斑狼疮（systemic lupus erythematosus，SLE）合并 Evans 综合征患者的临床特点。方法回顾性分析北京协和医院2004年1月至2012年7月 SLE 合并 Evans 患者的临床表现及实验室特点及治疗和预后。结果 SLE 并发 Evans 综合征患者22例，占同期 SLE 住院患者3400例的0．65％。其中男3例，女19例，平均35．1岁（16～53岁）。22例患者中以血液系统受累[特发性血小板减少性紫癜（idiopathic thrombocytopenic purpura，ITP）或自身免疫性溶血性贫血（autoimmune hemolytic anemia，AIHA）]为首发表现的11例（50％），确诊 SLE 后诊断 Evans 综合征者6例，二者同时诊断的5例。SLE 并发 Evans 综合征时，患者往往有多系统受累，表现为肾脏受累13例（59．1％），皮肤黏膜受累、关节炎各9例（40．9％），神经系统受累4例（18．2％），胃肠道、肺部受累各2例等。Evans 综合征多发生于 SLE 活动期，患者平均狼疮活动指数评分（11．45±7．6）分（3～30分）。伴发其他结缔组织病5例（22．7％）。经激素联合免疫抑制剂治疗后，20例好转，2例无效者应用利妥昔单抗后好转。结论 SLE 合并 Evans 综合征罕见，发生于 SLE 多系统受累及活动期。部分患者以ITP 或 AIHA 为 SLE 首发表现，应及时筛查多种自身抗体，并定期随访密切观察，以期早期诊治。
목적：총결계통성홍반랑창（systemic lupus erythematosus，SLE）합병 Evans 종합정환자적림상특점。방법회고성분석북경협화의원2004년1월지2012년7월 SLE 합병 Evans 환자적림상표현급실험실특점급치료화예후。결과 SLE 병발 Evans 종합정환자22례，점동기 SLE 주원환자3400례적0．65％。기중남3례，녀19례，평균35．1세（16～53세）。22례환자중이혈액계통수루[특발성혈소판감소성자전（idiopathic thrombocytopenic purpura，ITP）혹자신면역성용혈성빈혈（autoimmune hemolytic anemia，AIHA）]위수발표현적11례（50％），학진 SLE 후진단 Evans 종합정자6례，이자동시진단적5례。SLE 병발 Evans 종합정시，환자왕왕유다계통수루，표현위신장수루13례（59．1％），피부점막수루、관절염각9례（40．9％），신경계통수루4례（18．2％），위장도、폐부수루각2례등。Evans 종합정다발생우 SLE 활동기，환자평균랑창활동지수평분（11．45±7．6）분（3～30분）。반발기타결체조직병5례（22．7％）。경격소연합면역억제제치료후，20례호전，2례무효자응용리타석단항후호전。결론 SLE 합병 Evans 종합정한견，발생우 SLE 다계통수루급활동기。부분환자이ITP 혹 AIHA 위 SLE 수발표현，응급시사사다충자신항체，병정기수방밀절관찰，이기조기진치。
Objective To analyze the clinical features of Evans syndrome in systemic lupus erythematosus (SLE) patients.Methods Clinical and laboratory manifestations occurring during the disease course,as well as concomitant diseases,treatment and outcomes was carefully reviewed.Results There were 22 SLE patients with Evans Syndrome accounted for 0.65% of the 3 400 hospitalized SLE patients.Nineteen were female and 3 were male,with the mean age of 35.1 year.Evans syndrome was diagnosed prior to SLE in 11 patients,after SLE in 6 patients,and concomitantly with lupus in 5 patients.Active features of SLE were a frequent finding concomitant to Evans syndrome,including renal involvement in 13 cases (59.1%), arthritis in 9 patients (40.9%),rash in 6 patients (40.9%),neuropsychiatric manifestations in 4 cases (18.2%),etc.In addition,22.7% of our patients had an association with another autoimmune disease such as antiphospholipid syndrome.Conclusion Evans syndrome is a rare manifestation in SLE,occurring in patients with active and severe multisystemic SLE manifestations.Some lupus patients had ITP/AIHA onset,screening for auto-antibodies and regular follow-up are very helpful in the early diagnosis of SLE.