系统性红斑狼疮合并纯红细胞再生障碍性贫血1例报道及文献复习  

Systemic lupus erythematosus complicated with pure red cell aplasia:a case report and literature review

作　　者：陈琼[1] 方杰[2] 魏强华[1] CHEN Qiong;FANG Jie;WEI Qianghua(Department of Traditional Chinese Medicine,Shanghai General Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200080,China;Department of Laboratory,Shanghai General Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200080,China)

机构地区：[1]上海交通大学医学院附属第一人民医院中医科,上海200080 [2]上海交通大学医学院附属第一人民医院检验科,上海200080

出　　处：《上海交通大学学报（医学版）》2024年第2期278-286,共9页Journal of Shanghai Jiao tong University:Medical Science

基　　金：国家自然科学基金(82104908)。

摘　　要：该文对1例系统性红斑狼疮(systemic lupus erythematosus,SLE)合并纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)病例进行报道,并复习1974—2021年国内外文献报道的51例SLE合并PRCA患者的资料,对该52例患者(51+1例)的流行病学特征、临床特征、实验室指标、治疗以及预后进行统计分析。结果显示,52例患者多见于亚洲育龄期女性。诊断为SLE和诊断为PRCA时,患者的中位年龄分别为31.5岁和36.0岁。先诊断为SLE的患者,诊断为SLE与诊断为PRCA的间隔时间明显长于先诊断为PRCA的患者(P=0.042)。52例患者临床表现多为乏力、关节痛、雷诺现象和皮疹,并可合并自身免疫性溶血性贫血(autoimmune hemolytic anemia,AIHA)、胸腺瘤、甲状腺功能减退、重症肌无力(myasthenia gravis,MG)。实验室指标中抗核抗体(antinuclear antibody,ANA)阳性、抗双链DNA(double stranded DNA,dsDNA)抗体阳性、尿蛋白阳性和低补体水平的人数比例均较高。52例患者中,有51例的治疗方案包含糖皮质激素,占比98.08%,其次依次为输血治疗、环孢素A、环磷酰胺、高剂量静脉注射免疫球蛋白等。在报道了预后的50例病例中,预后好转44例,无效和死亡各3例。该文旨在提高临床医师对SLE合并PRCA的认识。This article reports a single case of a patient with systemic lupus erythematosus(SLE)combined with pure red cell aplasia(PRCA),and reviews 51 additional cases of patients reported by domestic and overseas papers from 1974 to 2021.These 52(51+1)cases were analyzed to summarize the epidemiological features,clinical features,laboratory inspections,treatments and prognosis of the patients.The results indicated that among all the 52 cases,cases of SLE combined with PRCA were mostly seen in Asian childbearing age women.The median ages of patients diagnosed with SLE and diagnosed with PRCA were 31.5 years and 36.0 years,respectively.The time interval between the initial diagnosis of SLE and subsequent diagnosis of PRCA was significantly longer than the interval for the initial diagnosis of PRCA,suggesting a delayed onset of SLE in these patients(P=0.042).Various clinical features of the 52 patients were reported,including mostly fatigue,joint pains,Raynaud phenomena and rashes,and SLE maybe combined with autoimmune hemolytic anemia(AIHA),thymoma,hypothyroidism and myasthenia gravis(MG).In these reported cases,laboratory indicators showed higher proportions of antinuclear antibody(ANA),anti double stranded DNA antibody(anti-dsDNA antibody),positive urinary protein and low complement levels.Among the 52 patients,51 cases(98.08%)were treated with glucocorticoids,followed by blood transfusion,cyclosporin A,cyclophosphamide and high-dose intravenous immunoglobulin.Of the 50 patients whose prognoses were reported,44 showed improvement,while 3 treatments were not effective and 3 resulted in death.This article aims to enhance the understanding of SLE combined with PRCA among doctors.

关 键 词：系统性红斑狼疮 纯红细胞再生障碍性贫血 流行病学特征 临床特征 

分 类 号：R593.24[医药卫生—内科学]