轻链型淀粉样变性肾损伤的临床特征及诊治情况分析  

Kidney injury caused by light chain amyloidosis:clinical features and treatment status

作　　者：吴晓静[1] 李欣[1] 张暮寅[1] 徐丽梨[1] 郝旭[1] 李灏[1] 王伟铭[1] WU Xiaojing;LI Xin;ZHANG Muyin;XU Lili;HAO Xu;LI Hao;WANG Weiming(Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 201801,China)

机构地区：[1]上海交通大学医学院附属瑞金医院肾脏内科,上海201801

出　　处：《上海医学》2022年第7期471-476,共6页Shanghai Medical Journal

基　　金：国家自然科学基金(82070740)。

摘　　要：目的探讨轻链型淀粉样变性(AL)肾损伤的临床特征,分析其诊治及预后情况,为临床诊治提供参考。方法回顾性分析2014年1月-2021年10月收治于上海交通大学医学院附属瑞金医院北部院区肾脏内科的34例轻链型AL及同期住院的11例非AL单克隆免疫球蛋白病(MGRS)肾损伤患者的临床资料,分别纳入轻链型AL组和非AL MGRS组。收集患者的一般人口学资料、基础疾病情况、器官受累情况、24 h尿蛋白定量、血肌酐、血尿酸、EPI公式估算的肾小球滤过率(EPI-eGFR)、血白蛋白、补体,以及M蛋白相关检查等。并对患者的肾脏疾病进行分期和评价疗效。结果纳入患者的初诊年龄为(58.3±8.4)岁,不同性别患者初诊年龄的差异无统计学意义(t=1.121,P=0.271)。患者初诊时单纯肾脏受累比例为64.7%(22/34),伴心脏受累比例为29.4%(10/34),伴消化道受累比例为5.9%(2/34)。不同性别初诊时肾脏预后分期的差异无统计学意义(χ^(2)=1.638,P=0.201)。轻链型AL组的血肌酐水平、血白蛋白水平、尿红细胞≥5/高倍镜视野(HP)患者比例、高血压患者比例均显著低于非AL MGRS组(Z=-3.645、-3.769,P值均<0.01;χ^(2)=6.433、8.779,P=0.011、0.003),而EPI-eGFR、24 h尿蛋白定量、血红蛋白水平、肾病综合征患者比例均显著高于非AL MGRS组(Z=-3.646、2.037、-2.841,P<0.01;χ^(2)=10.175,P=0.001)。轻链型AL患者血清免疫固定电泳(IFE)阳性率为70.6%,尿IFE阳性率为82.4%,血或尿IFE至少1项阳性的患者比例为88.2%;血清IFE类型以IgG-λ最常见(32.35%),其次为IgA-λ(17.65%),尿IFE类型以λ型最常见(76.47%)。血清游离轻链(FLC)比值异常率(>1.65或<0.26)为68.8%,如采用肾脏病FLC比值参考范围(>3.10或<0.37)则血清FLC比值异常率为81.3%。轻链型AL组患者中AL-λ型比例为88.2%(30/34),AL-κ型比例为11.8%(4/34)。AL-κ型患者的初诊年龄为(68.0±7.4)岁,显著高于AL-λ型的(57.0±7.7)岁(P=0.012)。AL-κ型相较AL-λ型患者�Objective To analyze clinical features and treatment status of kidney injury caused by light chain amyloidosis(AL),so as to provide evidence for diagnosis and treatment of the disease.Methods Clinical data of 34 patients with light chain AL and 11 patients with renal lesions of non-AL MGRS(monoclonal gammopathy with renal significance)hospitalized at renal department of Ruijin Hospital North District from January 2014 to October 2021 were enrolled in this retrospective study.The demographic information,basic diseases,organ involvement,24-hour urinary protein,serum creatinine,serum uric acid,estimated glomerular filtration rate by CKD-EPI formula(EPI-eGFR),serum albumin,complement and M protein were collected.The staging and curative effect of renal disease were evaluated.Results The average age of the patients at diagnosis was(58.3±8.4)years.There was no significant difference in the age at diagnosis between males and females(t=1.121,P=0.271).Only the kidney was involved in 64.7%(22/34)patients,29.4%(10/34)had heart involvement and 5.9%(2/34)had gastrointestinal involvement.There was no significant difference in renal prognostic stages between different genders(χ^(2)=1.638,P=0.201).Compared with the patients with non-AL MGRS,the patients with light chain AL had lower serum creatine(Z=-3.645,P<0.01),lower serum albumin(Z=-3.769,P<0.01),less hematuria(χ^(2)=6.433,P=0.011),less hypertension(χ^(2)=8.779,P=0.003),higher EPI-eGFR(Z=-3.646,P<0.01),higher 24-hour urinary protein(Z=2.037,P<0.01),higher hemoglobin(Z=-2.841,P<0.01)and more nephrotic syndrome(χ^(2)=10.175,P=0.001).Serum and urine immunofixation electrophoresis(IFE)results were positive in 70.6%and 82.4%of patients with light chain AL,respectively,and 88.2%of the patients had at least one positive IFE result(serum or urine).IgG-λwas the most common serum IFE type(32.35%),followed by IgA-λ(17.65%).Typeλwas the most common urinary IFE type(76.47%).The abnormal rate of serum FLC ratio was 68.8%(>1.65 or<0.26).When abnormal serum FLC ratio(renal reference

关 键 词：肾淀粉样变性 肾脏病理 临床表现 

分 类 号：R692[医药卫生—泌尿科学]