系统性红斑狼疮合并Evans综合征临床特点 |
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引用本文: | 王来芳,;赵清,;王立,;郑文洁,;赵岩,;曾小峰,;张奉春.系统性红斑狼疮合并Evans综合征临床特点[J].中华临床免疫和变态反应杂志,2014(3):170-173. |
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作者姓名: | 王来芳 ;赵清 ;王立 ;郑文洁 ;赵岩 ;曾小峰 ;张奉春 |
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作者单位: | [1]中国医学科学院北京协和医学院北京协和医院风湿免疫科、风湿免疫病学教育部重点实验室,北京100730; [2]河南大学淮河医院风湿免疫科进修医师,开封475000; [3]河南大学淮河医院风湿免疫科,开封475000 |
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基金项目: | 国家高技术研究发展计划(863计划),系统性红斑狼疮分子分型和个体化诊疗技术的研究(2012AA02A513),中华医学会基金(12040670367). |
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摘 要: | 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。
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关 键 词: | 系统性红斑狼疮 Evans 综合征 |
Clinical Features of Systemic Lupus Erythematosus Complicated with Evans Syndrome |
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Institution: | WANG Lai-fang, ZHAO Qing, WANG Li, ZHENG Wen-jie, ZHAO Yan, Zeng Xiao-feng, ZHANG Feng-chun (Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing 100730, China) |
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Abstract: | 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. |
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Keywords: | systemic lupus erythematosus Evans syndrome |
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