肾小球肾炎,膜性%M型磷脂酶A2受体%IgG亚型
腎小毬腎炎,膜性%M型燐脂酶A2受體%IgG亞型
신소구신염,막성%M형린지매A2수체%IgG아형
Glomerulonephritis,Membranous%M-type phospholipase A2 receptor%IgG subclass
目的 通过检测不明原因的不典型膜性肾病(UAMN)患者血清中M型磷脂酶A2受体(PLA2R)抗体及肾组织中IgG亚型的分布,研究UAMN患者血清中PLA2R抗体及肾组织中IgG亚型的分布特点.方法 入选2004年2月至2011年11月于北京大学人民医院肾内科诊断为UAMN的患者20例.对照组包括特发性膜性肾病(IMN)患者20例,继发性膜性肾病(SMN)患者13例.其中SMN组中包括狼疮膜性肾病(LMN)5例,乙肝病毒相关性膜性肾病(HBV-MN)8例.分析入选患者的临床及病理资料,检测血清PLA2R抗体及肾组织中IgG亚型的分布特点.结果 (1) UAMN组患者肾活检时平均年龄为(37.9±3.8)岁,IMN组为(50.1±3.0)岁,SMN组为(49.5±4.5)岁,其中UAMN组平均年龄显著低于IMN组(P=0.0178).UAMN组、IMN组和SMN组的女/男比例分别为0.8:1、0.7∶1和0.6∶1,差异无统计学意义.(2)与SMN组比较,UAMN组24 h尿蛋白定量更低(3.47g比7.89g,P=0.023),大量蛋白尿患者的比例也更低(50.0％比84.6％,P=0.043),血清IgG水平更低[(8.40±3.58) g/L比(10.09±4.69) g/L,P=0.025],血清ANA阳性率也更低(10.0％比53.8％,P=0.006).人血白蛋白、血肌酐、eGFR、血清HBsAg阳性率、血清HBeAg阳性率、血清抗HCV阳性率以及低白蛋白血症、肾病综合征患者比例在3组中的差异均无统计学意义.(3)肾脏病理免疫荧光结果示,与IMN组比较,UAMN组IgA、IgM和C1q荧光阳性率更高,差异有统计学意义(均P＜ 0.01);与SMN组比较,则两组的IgA、IgG、IgM、C1q和C3荧光阳性率及荧光强度差异均无统计学意义.(4)在所有53例患者血清中,仅有10例IMN组患者血清抗PLA2R自身抗体检测为阳性,在IMN组PLA2R自身抗体检出率为50％.3组间的PLA2R自身抗体检出率差异有统计学意义(P＜0.01).(5)对肾组织IgG亚型的检测:UAMN组中IgG1亚型阳性率最高(40％),IgG3亚型阳性率最低(15％).IMN组中,IgG4亚型阳性率最高(60％),其次为IgG1(15％),IgG2及IgG3亚型阳性率最低(均为5％).SMN组中,IgG1亚型阳性率最高(76.9％),IgG4亚型阳性率最低(30.8％).UAMN组IgG1、IgG3亚型阳性率与IMN及SMN组比较差异均无统计学,IgG2亚型阳性率表现出与SMN组差异有统计学意义(30.0％比69.2％,P＜0.05),IgG4亚型阳性率与IMN组差异有统计学意义(20％比60％,P＜0.05).SMN组IgG1、IgG2和IgG3亚型的阳性率均显著高于IMN组(分别为76.9％、69.2％、46.2％比15％、5％、5％,均P＜0.01).IMN组IgG4亚型在肾小球沉积的阳性率高于SMN及UAMN组,但仅与UAMN组差异具统计学意义(P=0.0225).UAMN组显示出了与IMN及SMN组的不同,更类似于SMN组特点.结论 UAMN组患者抗PLA2R自身抗体检测均为阴性,肾组织以IgG1亚型沉积为主,表现了类似SMN的特点,但在临床指标上又显示了与SMN的不同.因此要加强临床的长期随访,深入对这一类疾病的研究.
目的 通過檢測不明原因的不典型膜性腎病(UAMN)患者血清中M型燐脂酶A2受體(PLA2R)抗體及腎組織中IgG亞型的分佈,研究UAMN患者血清中PLA2R抗體及腎組織中IgG亞型的分佈特點.方法 入選2004年2月至2011年11月于北京大學人民醫院腎內科診斷為UAMN的患者20例.對照組包括特髮性膜性腎病(IMN)患者20例,繼髮性膜性腎病(SMN)患者13例.其中SMN組中包括狼瘡膜性腎病(LMN)5例,乙肝病毒相關性膜性腎病(HBV-MN)8例.分析入選患者的臨床及病理資料,檢測血清PLA2R抗體及腎組織中IgG亞型的分佈特點.結果 (1) UAMN組患者腎活檢時平均年齡為(37.9±3.8)歲,IMN組為(50.1±3.0)歲,SMN組為(49.5±4.5)歲,其中UAMN組平均年齡顯著低于IMN組(P=0.0178).UAMN組、IMN組和SMN組的女/男比例分彆為0.8:1、0.7∶1和0.6∶1,差異無統計學意義.(2)與SMN組比較,UAMN組24 h尿蛋白定量更低(3.47g比7.89g,P=0.023),大量蛋白尿患者的比例也更低(50.0％比84.6％,P=0.043),血清IgG水平更低[(8.40±3.58) g/L比(10.09±4.69) g/L,P=0.025],血清ANA暘性率也更低(10.0％比53.8％,P=0.006).人血白蛋白、血肌酐、eGFR、血清HBsAg暘性率、血清HBeAg暘性率、血清抗HCV暘性率以及低白蛋白血癥、腎病綜閤徵患者比例在3組中的差異均無統計學意義.(3)腎髒病理免疫熒光結果示,與IMN組比較,UAMN組IgA、IgM和C1q熒光暘性率更高,差異有統計學意義(均P＜ 0.01);與SMN組比較,則兩組的IgA、IgG、IgM、C1q和C3熒光暘性率及熒光彊度差異均無統計學意義.(4)在所有53例患者血清中,僅有10例IMN組患者血清抗PLA2R自身抗體檢測為暘性,在IMN組PLA2R自身抗體檢齣率為50％.3組間的PLA2R自身抗體檢齣率差異有統計學意義(P＜0.01).(5)對腎組織IgG亞型的檢測:UAMN組中IgG1亞型暘性率最高(40％),IgG3亞型暘性率最低(15％).IMN組中,IgG4亞型暘性率最高(60％),其次為IgG1(15％),IgG2及IgG3亞型暘性率最低(均為5％).SMN組中,IgG1亞型暘性率最高(76.9％),IgG4亞型暘性率最低(30.8％).UAMN組IgG1、IgG3亞型暘性率與IMN及SMN組比較差異均無統計學,IgG2亞型暘性率錶現齣與SMN組差異有統計學意義(30.0％比69.2％,P＜0.05),IgG4亞型暘性率與IMN組差異有統計學意義(20％比60％,P＜0.05).SMN組IgG1、IgG2和IgG3亞型的暘性率均顯著高于IMN組(分彆為76.9％、69.2％、46.2％比15％、5％、5％,均P＜0.01).IMN組IgG4亞型在腎小毬沉積的暘性率高于SMN及UAMN組,但僅與UAMN組差異具統計學意義(P=0.0225).UAMN組顯示齣瞭與IMN及SMN組的不同,更類似于SMN組特點.結論 UAMN組患者抗PLA2R自身抗體檢測均為陰性,腎組織以IgG1亞型沉積為主,錶現瞭類似SMN的特點,但在臨床指標上又顯示瞭與SMN的不同.因此要加彊臨床的長期隨訪,深入對這一類疾病的研究.
목적 통과검측불명원인적불전형막성신병(UAMN)환자혈청중M형린지매A2수체(PLA2R)항체급신조직중IgG아형적분포,연구UAMN환자혈청중PLA2R항체급신조직중IgG아형적분포특점.방법 입선2004년2월지2011년11월우북경대학인민의원신내과진단위UAMN적환자20례.대조조포괄특발성막성신병(IMN)환자20례,계발성막성신병(SMN)환자13례.기중SMN조중포괄랑창막성신병(LMN)5례,을간병독상관성막성신병(HBV-MN)8례.분석입선환자적림상급병리자료,검측혈청PLA2R항체급신조직중IgG아형적분포특점.결과 (1) UAMN조환자신활검시평균년령위(37.9±3.8)세,IMN조위(50.1±3.0)세,SMN조위(49.5±4.5)세,기중UAMN조평균년령현저저우IMN조(P=0.0178).UAMN조、IMN조화SMN조적녀/남비례분별위0.8:1、0.7∶1화0.6∶1,차이무통계학의의.(2)여SMN조비교,UAMN조24 h뇨단백정량경저(3.47g비7.89g,P=0.023),대량단백뇨환자적비례야경저(50.0％비84.6％,P=0.043),혈청IgG수평경저[(8.40±3.58) g/L비(10.09±4.69) g/L,P=0.025],혈청ANA양성솔야경저(10.0％비53.8％,P=0.006).인혈백단백、혈기항、eGFR、혈청HBsAg양성솔、혈청HBeAg양성솔、혈청항HCV양성솔이급저백단백혈증、신병종합정환자비례재3조중적차이균무통계학의의.(3)신장병리면역형광결과시,여IMN조비교,UAMN조IgA、IgM화C1q형광양성솔경고,차이유통계학의의(균P＜ 0.01);여SMN조비교,칙량조적IgA、IgG、IgM、C1q화C3형광양성솔급형광강도차이균무통계학의의.(4)재소유53례환자혈청중,부유10례IMN조환자혈청항PLA2R자신항체검측위양성,재IMN조PLA2R자신항체검출솔위50％.3조간적PLA2R자신항체검출솔차이유통계학의의(P＜0.01).(5)대신조직IgG아형적검측:UAMN조중IgG1아형양성솔최고(40％),IgG3아형양성솔최저(15％).IMN조중,IgG4아형양성솔최고(60％),기차위IgG1(15％),IgG2급IgG3아형양성솔최저(균위5％).SMN조중,IgG1아형양성솔최고(76.9％),IgG4아형양성솔최저(30.8％).UAMN조IgG1、IgG3아형양성솔여IMN급SMN조비교차이균무통계학,IgG2아형양성솔표현출여SMN조차이유통계학의의(30.0％비69.2％,P＜0.05),IgG4아형양성솔여IMN조차이유통계학의의(20％비60％,P＜0.05).SMN조IgG1、IgG2화IgG3아형적양성솔균현저고우IMN조(분별위76.9％、69.2％、46.2％비15％、5％、5％,균P＜0.01).IMN조IgG4아형재신소구침적적양성솔고우SMN급UAMN조,단부여UAMN조차이구통계학의의(P=0.0225).UAMN조현시출료여IMN급SMN조적불동,경유사우SMN조특점.결론 UAMN조환자항PLA2R자신항체검측균위음성,신조직이IgG1아형침적위주,표현료유사SMN적특점,단재림상지표상우현시료여SMN적불동.인차요가강림상적장기수방,심입대저일류질병적연구.
Objective To investigate the characteristic of autoantibodies of M-type phospholipase A2 receptor (PLA2R) in serum and the glomerular IgG subclass deposits in undetermined atypical membranous nephropathy (MN) patients.Methods From Feb 2004 to Nov 2011,53 cases diagnosed as MN by kidney puneture biopsy in our hospital were included into the study.There were 20 undetermined atypical membranous nephropathy (UAMN),20 idiopathic membranous nephropathy (IMN) and 13 secondary membranous nephropathy (SMN) which were composed of lupus membranous nephropathy (LMN) and HBV related membranous nephropathy (HBV-MN).Clinlical and pathological characteristics were analyzed.The autoantibodies of PLA2R in serum were detected and the glomerular IgG subclass deposits were observed.Results (1) The average age underwent renal biopsy was (37.9±3.8) years of UAMN,(50.1±3.0) years of IMN and (49.5±4.5) years of SMN.The difference in onset average age at disease was significant between UAMN and IMN (P =0.0178).The female/male ratio (F/M) in UAMN,IMN and SMN was 0.8∶ 1,0.7∶1 and 0.6∶ 1(P ＞ 0.05).(2) Compared with SMN,the level of 24-hours urinary protein excretion (3.47 g vs 7.89 g,P =0.023),the ratio of amount urinary protein patients (50.0％ vs 84.6％,P=0.043),the level of serum IgG [(8.40±3.58) g/L vs (10.09±4.69) g/L,P =0.025] and the positive rate of ANA in serum (10.0％ vs 53.8％,P =0.006) in UAMN were all much lower.There were no significant statistical differences in serum albumin,serum creatinine,eGFR,positive rate of HBsAg,HBeAg or HCV,as well as the ratio of hypo-albuminemia and nephrotic syndrome among the three groups.(3) IF positive rate of IgA,IgM and C1q in UAMN were all significantly higher than that in IMN (P ＜ 0.01).There were no significant differences in IF positive rate of IgA,IgM,C1q,IgG and C3 between UAMN and SMN.The IF strength of IgA,IgG,IgM,C3 and C1q in UAMN showed no significant differences between UAMN and SMN.(4) The serum autoantibodies of PLA2R were only detected in 10 cases of IMN group (50％) with all the other cases negative.This detection rate of serum autoantibodies of PLA2R showed significant statistical differences among the three groups (P ＜ 0.01),but no differences between UAMN and SMN (the detection rate in both groups were 0％).(5) IgG1 deposits was the dominant IgG on the glomeruli in UAMN group (40％),as well as in SMN group (76.9％).IgG4 deposits was the dominant IgG on the glomeruli in IMN group (60％).The positive rate of IgG1 and IgG3 in UAMN showed no significant statistical differences when compared with IMN or SMN.The positive rate of IgG2 in UAMN was significantly lower than in SMN (30.0％ vs 69.2％,P ＜ 0.05).The positive rate of IgG4 in UAMN was significantly lower than in IMN (20％ vs 60％,P ＜ 0.05).The positive rate of IgG1,IgG2 and IgG3 in SMN were all significantly higher than in IMN.Conclusions None of the UAMN group had autoantibodies of PLA2R in serum,and IgG1 deposits was the dominant IgG subclass on the glomeruli which indicated the similarity with the SMN group.At the same time,UAMN was significantly different from SMN in clinical manifestations.
