系统性红斑狼疮合并Evans综合征的临床特征分析  

Clinical Features of Systemic Lupus Erythematous Complicated with Evans Syndrome

作　　者：王丛丛 王卫敏[1] 田文亮[1] 王菲[1] 孙慧[1] 孙玲[1] WANG Congcong;WANG Weimin;TIAN Wenliang;WANG Fei;SUN Hui;SUN Ling(Department of Hematology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院血液科,河南郑州450052

出　　处：《河南医学研究》2021年第34期6349-6353,共5页Henan Medical Research

摘　　要：目的观察分析系统性红斑狼疮(SLE)合并Evans综合征(ES)患者的临床特征。方法回顾性分析2015年5月至2020年5月郑州大学第一附属医院收治的SLE-Evans患者的临床表现、实验室检查结果、影像学检查结果、治疗及转归情况。结果SLE-Evans患者5例,其中女4例,男1例;中位年龄48岁;3例患者以ES为首发表现,1例患者以SLE为首发表现,1例患者SLE与ES症状同时发生。SLE-Evans患者常常有多个系统受累,本研究中消化系统受累3例,肾受累、心脏受累、多浆膜腔积液、面部红斑各2例,肺受累、神经系统受累、口腔溃疡、关节炎各1例,伴发其他自身免疫性疾病3例。经激素联合免疫抑制剂治疗后4例好转,1例死亡。结论成人SLE-Evans罕见,常常合并多个器官、多个系统且危及患者生命的并发症,易伴发其他自身免疫性疾病。Objective To observe and analyze the clinical features of patients with systemic lupus erythematous(SLE)complicated with Evans syndrome(ES).Methods The clinical manifestations,laboratory examination,imaging examination,treatment and prognosis of SLE-Evans patients admitted to the First Affiliated Hospital of Zhengzhou University from May 2015 to May 2020 were analyzed retrospectively.Results There were 5 patients with SLE-Evans,including 4 females and 1 male,with a median age of 48 years.Three patients had ES as the first manifestation,one patient presented with SLE initially,one patient suffered from both SLE and ES at the same time.Multiple system involvement are frequently observed in SLE-Evans,including digestive system involvement in 3 cases,lupus nephritis,cardiac involvement,polyserous effusion and facial erythema in 2 cases each,pulmonary and neuropsychiatric involvement,oral ulcer and arthritis in 1 case each,with other autoimmune diseases in 3 patients.There were four patients achieved improvement following treatment of glucocorticoids and immunosuppressants,whereas 1 patient died.Conclusion Adult SLE-Evans is a rare disease that is often associated with life-threatening complications in multiple organs and systems.It is easy to be accompanied by other autoimmune diseases.

关 键 词：系统性红斑狼疮 EVANS综合征 疾病特征 

分 类 号：R593.24[医药卫生—内科学]