有肾脏意义的单克隆免疫球蛋白病相关肾损伤的临床特征  

Clinical features of monoclonal gammopathy of renal significance related renal lesions

作　　者：吴晓静[1] 李欣[1] 张暮寅[1] 徐丽梨[1] 郝旭[1] 王伟铭[1] Wu Xiao-jing;Li Xin;Zhang Mu-yin;Xu Li-li;Hao Xu;Wang Wei-ming(Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院,上海200025

出　　处：《临床肾脏病杂志》2022年第8期624-629,共6页Journal Of Clinical Nephrology

摘　　要：目的通过分析有肾脏意义的单克隆免疫球蛋白病(monoclonal gammopathy of renal significance,MGRS)相关肾损伤的临床特点和病理类型,探索有助于MGRS相关肾损伤鉴别诊断的临床指标。方法采用回顾性研究方法,收集2014年1月至2021年2月在上海交通大学医学院附属瑞金医院北部院区肾脏科住院的经病理确诊为MGRS相关肾损伤的患者的临床资料,分析MGRS相关肾损伤各病理类型的临床特征。同时选取同期住院的意义未明的单克隆免疫球蛋白病(monoclonal gammopathy of undetermined significance,MGUS)合并肾脏病的患者作为对照组,比较MGRS相关肾损伤与MGUS合并肾脏病在临床特征及血液学指标方面的差异。结果本组44例MGRS肾损伤的肾脏病理类型包括:淀粉样变性肾损害34例、轻链沉积病3例、增生性肾小球肾炎伴单克隆免疫球蛋白沉积2例、管型肾病2例、血栓性微血管病1例、冷球蛋白性肾小球肾炎1例、纤维性肾小球肾炎1例。与非淀粉样变的MGRS肾损伤相比,淀粉样变组的肾病综合征占比显著较高(85.3%比40.0%,P=0.008),而高血压占比显著较低(23.5%比80.0%,P=0.002)。淀粉样变肾损害的单克隆完整免疫球蛋白最常见为IgG(约占61.9%),轻链类型以λ为主(约占89.7%),而轻链沉积病的轻链类型以κ多见。与同期住院的21例MGUS合并肾脏病患者相比,MGRS组的血免疫固定电泳阳性率低于MGUS组(70.5%比100%,P=0.005),两组间尿免疫固定电泳阳性率差异无统计学意义(P=0.195),MGRS组血游离轻链(free light chain,FLC)比值异常率高于MGUS组(63.2%比25.0%,P=0.038),MGRS组的低补体血症比例高于MGUS组(40.9%比14.3%,P=0.032)。血FLC比值异常用于诊断MGRS的敏感度为63.2%、特异度为75%,总符合率为67.7%。骨髓流式细胞术检测结果显示,异常浆细胞比例诊断MGRS的AUC及95%CI为0.855(0.719~0.991),评价骨髓流式细胞术检测得出的异常浆细胞比例≥0.55%用于诊断MGRS的敏�Objective To explore the clinical features and pathological spectrum of monoclonal gammopathy of renal significance(MGRS)related renal lesions and search for valuable clinical parameters aiding in their differential diagnoses.Methods From January 2014 to February 2021,medical records and clinicopathological data were reviewed for MGRS patients with associated renal lesions as determined by renal pathology.Monoclonal gammopathy of undetermined significance(MGUS)patients complicated with kidney disease hospitalized during the same period were selected as control group.Results MGRS associated renal lesions included light chain amyloidosis(n=34),light chain deposition disease(n=3),proliferative glomerulonephritis with monoclonal Ig deposits(PGNMID,n=2),cast nephropathy(n=2),thrombotic microangiopathy(TMA,n=1),cryoglobulin glomerulonephritis(n=1)and fibroglomerulonephritis(n=1).Patients with amyloidosis significantly had a lower proportion of hypertension(23.5%vs 80.0%,P=0.002)and a higher proportion of nephrotic syndrome(85.3%vs 40.0%,P=0.008)than nonamyloidotic MGRS group.In light chain amyloidosis,the types of serum intact Ig were predominantly IgG(61.9%)and a major type of serum light chain wasλ(89.7%).In LCDD,a major type of serum light chain was k.As compared with MGUS group,MGRS group had a lower positive rate of serum immunofixation electrophoresis(IFE,70.5%vs 100%,P=0.005),a higher abnormal rate of serum free light chain(FLC)ratio(63.2%vs 25.0%,P=0.038)and a higher proportion of hypocomplementaemia(40.9%vs 14.3%,P=0.032).No significant inter-group difference existed in positive rate of urinary IFE(P=0.195).The sensitivity of abnormal FLC ratio for diagnosing MGRS was 63.2%with a specificity of 75%and overall accuracy of 67.7%.AUC(area under curve)and 95%CI of abnormal plasma cell ratio in BM for diagnosing MGRS were 0.855(0.719-0.991).The sensitivity,specificity and overall accuracy of abnormal plasma cell ratio≥0.55%were 83.3%,80%and 82.5%respectively.Conclusion The most common pathological type of MGRS r

关 键 词：单克隆免疫球蛋白病 肾脏疾病 肾脏病理 

分 类 号：R692[医药卫生—泌尿科学]