血清IgG4检测在IgG4相关胆管炎临床诊断及鉴别诊断的初步应用

为探讨自身抗体和血清IgG4对IgG4相关性胆管炎(IgG4associated cholangitis,IgG4-SC)的诊断及鉴别诊断的临床意义,选取42例IgG4-SC患者、168例疾病对照组患者及43例健康对照,采用免疫散射比浊法检测IgG4水平;采用间接免疫荧光法检测抗核抗体(anti-nuclear antibody,ANA)、抗中性粒细胞胞质抗体(anti-neutrophil antibody,ANCA)、抗平滑肌抗体(anti-smooth muscle antibody,SMA)和抗线粒体抗体(anti-mitochondrial antibody,AMA),并对结果进行统计分析。结果如下:(1)IgG4-SC组ANA阳性率最高为40.48%,SMA最低为0,除ANA阳性率外,其余自身抗体检测结果均显著低于疾病对照组,组间差异均具有非常显著统计学意义(P0.01)。(2)血清IgG4升高与PSC、AIH、PBC及正常对照组相比,差异具有非常显著统计学意义(P0.01);其ROC AUC=0.9640.7(P0.01)。由此得出,自身抗体和血清IgG4的高表达,对于提高IgG4-SC诊断及其与自身免疫性肝病鉴别诊断的准确性有重要临床价值。     

161例自身免疫性肝病患者相关自身抗体检测的临床研究

目的探讨自身抗体检测对自身免疫性肝病(AILD)诊断的临床意义。方法 161例自身免疫性肝病[其中自身免疫性肝炎(AIH)68例、原发性胆汁性肝硬化(PBC)41例和原发性硬化性胆管炎(PSC)52例]、276例病毒性肝炎患者和50例健康体检者采用间接免疫荧光法(IIF)检测抗核抗体(ANA)、抗中性粒细胞胞浆抗体(ANCA)、抗平滑肌抗体(SMA)和抗线粒体抗体(AMA)等自身抗体,采用酶联免疫吸附法(ELISA)检测抗MPO抗体,并对其结果进行回顾性分析。结果 161例AILD检测ANA、ANCA、SMA、抗MPO抗体及AMA结果显示其阳性率分别为42.9%、46.6%、29.2%、30.1%、42.2%,与病毒性肝炎及对照组比较,均P<0.01。各种肝病对自身抗体的检测结果显示AIH较高;AIH中ANCA及p-ANCA检出率与其他肝病组及对照组相比,除PSC外P<0.01,有非常显著意义。结论肝病相关自身抗体的联合检测对自身免疫性肝病的检出、诊断、鉴别诊断、临床分型有重要临床价值,对提高自身免疫性肝病在临床上同病毒性肝炎鉴别诊断和指导治疗有着非常重要的意义。     

原发性硬化性胆管炎自身抗体检测的临床意义

目的探讨自身抗体检测对诊断原发性硬化性胆管炎(PSC)患者的临床意义。方法采用间接免疫荧光法(IIF)检测抗核抗体(ANA)和抗中性粒细胞胞质抗体(ANCA)并对其结果进行回顾性分析。结果 PSC检测ANA、ANCA、c-ANCA和p-ANCA结果与对照组比较P<0.01,p-ANCA与c-ANCA组间比较P<0.01,差异有非常显著性意义。PSC患者与病毒性肝炎比较,除HCV以外,经χ2检验,P<0.01差异均有非常显著性意义。结论血清ANCA、p-ANCA抗体检测对诊断PSC有着重要作用。对提高PSC在临床上同其它肝病鉴别诊断亦非常重要。     

中国西部自身免疫性肝病患者自身抗体的分布特征

目的: 探讨中国西部自身免疫性肝病患者中相关自身抗体的存在状况及特征.方法: 57例自身免疫性肝病患者分为3组: 自身免疫性肝炎(AIH)12例、原发性胆汁性肝硬化(PBC)32例、原发性硬化性胆管炎(PSC)13例.用间接免疫荧光法检测抗核抗体(ANA)、平滑肌抗体(SMA)、抗肝肾微粒抗体1型抗体(anti-LKM1)、抗线粒体抗体(AMA)和抗中性粒细胞胞质抗体(ANCA),Western blot检测抗肝细胞胞溶质抗原1型抗体(anti-LC1)、抗可溶性肝抗原/肝胰抗原抗体(anti-SLA/LP)、抗肝肾微粒抗体1型(anti-LKM1)、 AMA-M2亚型等多种肝抗原自身抗体.结果: 57例中ANA、AMA、M-2、pANCA阳性率在组间有统计学差异(P<0.01).PBC中AMA、M-2阳性检出率均为100%, PSC中pANCA阳性检出率为53.8%, Fisher精确检验在α'=0.002水准与其他各组比较有统计学差异.AIH与PBC的ANA阳性率分别为100%和50%,Fisher精确检验在α'=0.002水准二者无统计学意义,与其他各组比较有明显差异.在AIH组SMA阳性率为25%,LKM-1、LC-1、SLA/LP阳性率均为8.3%, 与其他组无统计学意义,可能与病例少有关.PBC中分别有1例患者ANA、SMA以及ANA、LKM-1同时阳性, PSC中有1例ANA、SLA/LP同时阳性,此3例患者结合性别、生化、自身抗体等资料符合AIH诊断条件;AIH中有1例M-2阳性综合各项资料符合PBC(重叠综合征).结论: 肝抗原自身抗体、ANA、AMA及M-2亚型的检测有助于自身免疫性肝病的诊断.对肝炎病毒血清标志物阴性的肝功能异常者应该行肝抗原自身抗体检测协助诊断.     

原发性胆汁肝硬化患者血清中抗线粒体抗体及其亚型和抗核抗体的联合检测的临床价值

为了分析原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者的抗线粒体抗体(anti-mitochondria antibody,AMA)及其M2、M4、M9亚型、抗核抗体(antinuclear antibody,ANA)的阳性率。应用间接免疫荧光法检测ANA、AMA,免疫斑点法检测AMA M2、M4、M9。结果显示91例PBC患者中AMA有89例为阳性。其中96.7%(88/91)的患者M2型阳性,45.1%(41/91)M4型阳性,2.2%(2/91)M9型阳性。39/91例患者ANA阳性,其中21例为核膜型。ANA、AMA及其M2、M4、M9亚型联合检测对于PBC患者的诊断有重要价值。     

自身免疫性肝炎和乙型肝炎患者自身抗体检测及对比分析

目的:检测分析自身免疫性肝炎(AIH)与乙型肝炎(HB)患者血清自身抗体特点及诊断应用价值。方法:采用间接免疫荧光法检测AIH组(n=43)和HB组(n=100)血清抗核抗体(ANA)、抗平滑肌抗体(SMA)、抗线粒体抗体(AMA)及抗中性粒细胞胞浆抗体(pANCA、cANCA),比较两组自身抗体检出率、ANA滴度、荧光模型及肝功能和免疫球蛋白。结果:AIH组自身抗体以ANA、SMA为主,阳性率分别为93.02和67.44%,两种抗体同时阳性的检出率为55.80%,AMA及pANCA、cANCA的检出率分别为13.95%和6.97%、2.32%;而HB组只检测出16例(16.0%)ANA,无一例SMA阳性。AIH组ANA以高滴度(≥1:320)抗体为主,荧光核型主要以核仁、核膜型为主,丙氨酸氨基转移酶(ALT)、天门冬氨酸氨基转移酶(AST)及IgG含量明显高于HB组。HB组ANA以低滴度(≤1:100)抗体为主,荧光核型以颗粒型所占比例较高。结论:检测AIH和HB患者自身抗体相关指征对提高诊断准确性,制定治疗方案有重要作用。     

542例O型血孕妇血清中IgG抗A(B)抗体效价结果分析

目的了解O型血孕妇血清中IgG抗A(B)抗体效价阳性率。方法采用间接抗人球法检测IgG抗A(B)抗体效价。结果检测效价在1∶64以上者达16.7%,且O-A组O-B组阳性率无显著性差异(P>0.05,χ2=1.72)。结论检测孕妇血清中IgG类抗体效价,≥1∶64者及时治疗,可预防和减低新生儿溶血病的发生。     

原发性胆汁性肝硬化患者抗线粒体抗体亚型检测的临床意义

为探讨原发性胆汁性肝硬化(PBC)患者血清抗线粒体抗体(AMA)亚型抗M2、M4、M9抗体的临床意义,采用间接免疫荧光法检测96例PBC患者血清AMA水平,采用酶免疫斑点法检测AMA-M2、M4、M9抗体水平,应用全自动生化分析仪检测ALT等生化指标。结果表明96例PBC患者中,荧光法AMA阳性率为84.4%,抗M2抗体为81.3%;斑点法抗M2抗体阳性率为72.9%,抗M4抗体为44.8%,抗M9抗体为18.8%,抗M2和M4抗体同时阳性阳性率为43.8%,抗M2和M9抗体同时阳性阳性率为16.7%,抗M2、M4和M9抗体同时阳性阳性率为13.5%,抗M4抗体阳性的PBC患者ALT、AST和IgM水平明显高于抗M4抗体阴性(P<0.05),抗M9抗体阳性的PBC患者ALT、AST和IgG水平明显低于抗M9抗体阴性(P<0.05)。AMA-M2抗体的检测对PBC患者有诊断意义,抗M4抗体和抗M9抗体的检测对于PBC患者的病情判断有意义。     

特发性膜性肾病患者血清抗磷脂酶A2受体抗体与尿中IgG4检测的临床意义

目的：探讨特发性膜性肾病（IMN）患者血清抗磷脂酶A2受体（PLA2R）抗体及尿IgG4检测的临床意义。方法：将90例膜性肾病（MN）患者按照病理类型分为特发性膜性肾病（IMN）组（52例）和继发性膜性肾病（SMN）组（38例）;另选同期体检健康者35例作为对照组。检测各组血清抗PLA2R抗体表达和尿IgG4水平;分析治疗后不同转归的IMN患者之间的血清抗PLA2R抗体阳性率及尿IgG4水平差异。结果：IMN组的血清抗PLA2R抗体阳性率及尿IgG4水平均明显高于SMN组和对照组（P＜0.05）;SMN组的尿IgG4水平明显高于对照组（P＜0.05）,而血清抗PLA2R抗体阳性率与对照组差异无显著统计学意义（P＞0.05）;治疗后,IMN缓解组的血清抗PLA2R抗体阳性率明显低于未缓解组（P＜0.05）,治疗后尿IgG4水平较本组治疗前及未缓解组治疗后均降低（P＜0.05）,而治疗后未缓解组的尿IgG4水平较治疗前不降反升（P＜0.05）;IMN复发患者的血清抗PLA2R抗体阳性率及尿IgG4水平均明显高于无复发者（P＜0.05）。结论：IMN患者血清抗PLA2R抗体阳性率和尿IgG4水平明显升高,其变化与IMN的病情及远期结局均有关,二者联合检测有助于IMN的诊断、病情活动及预后评估。     

血清IgG4水平对常见自身免疫性疾病的鉴别诊断价值北大核心CSCD

目的:探讨血清IgG4水平在鉴别诊断IgG4相关性疾病(IgG4-RD)与其他自身免疫性疾病中的应用价值。方法:选择2014年9月至2021年3月于南京医科大学附属淮安第一医院、苏北人民医院及徐州市第一人民医院住院治疗并确诊的IgG4-RD患者26例,自身免疫性疾病患者549例,包括系统性红斑狼疮83例,类风湿关节炎80例,干燥综合征188例,强直性脊柱炎21例,进行性系统性硬化症10例,结缔组织病126例,成人Still病5例,白塞氏病3例,皮肌炎28例,风湿性多肌痛症5例。另选取50例健康体检者作为对照组。使用BNII特定蛋白分析仪检测各组样本血清IgG4水平。通过ROC曲线评价血清IgG4鉴别诊断IgG4-RD与其他自身免疫性疾病的敏感度和特异度。结果:IgG4-RD组血清IgG4水平明显高于其他各自身免疫性疾病组和健康对照组,差异有统计学意义(P<0.001)。分别以血清IgG4≥1.35 g/L和IgG4≥2.01 g/L作为诊断IgG4-RD的临界值,IgG4-RD组的阳性率明显高于自身免疫性疾病组和健康对照组,差异有统计学意义(P<0.001)。以血清IgG4≥1.35 g/L为正常参考值上限,IgG4-RD组样本血清IgG4水平及IgG4在总IgG中所占比例均高于非IgG4-RD阳性样本,差异有统计学意义(P<0.001)。血清IgG4水平用于鉴别诊断IgG4-RD的ROC曲线显示,以IgG4≥2.07 g/L作为诊断临界值时,敏感度为96.15%,特异度为92.57%,曲线下面积(AUC)为0.979。结论:非IgG4-RD自身免疫性疾病也可出现血清IgG4水平升高现象,以血清IgG4≥2.07 g/L作为诊断临界值能提升对IgG4-RD与非IgG4-RD自身免疫性疾病的鉴别诊断效能,但仍需在临床中进一步验证。     

Organ and non-organ specific autoantibody titres and IgG levels as markers of disease activity: a longitudinal study in childhood autoimmune liver disease

No longitudinal study has investigated whether autoantibody titres and serum IgG levels correlate with disease activity in autoimmune liver disease. To determine this, we investigated prospectively 19 patients on 254 occasions between 10 months to 5 years from diagnosis. Nine had anti-nuclear and/or anti-smooth muscle antibody (ANA/SMA) positive autoimmune hepatitis (type 1 AIH), 5 liver kidney microsomal type 1 (LKM-1) positive AIH (type 2 AIH) and 5 ANA/SMA positive autoimmune sclerosing cholangitis (ASC). Correlation between IgG levels, titres of ANA, SMA and LKM-1 and levels of the organ specific autoantibodies anti-liver specific protein (anti-LSP), and anti-asialoglycoprotein receptor (anti-ASGPR) with biochemical evidence of disease activity, as measured by serum aspartate amino transferase (AST) levels, was sought during the course of the disease. AST levels correlated with levels of anti-LSP, anti-ASGPR and IgG in type 1 and 2 AIH, but not in ASC. Positive correlation with AST was also observed for LKM-1 titres in type 2 AIH and for SMA titres in type 1 AIH, but not in ASC. In both AIH and ASC, AST levels correlated with the T cell-dependent immune responses anti rubella IgG and anti tetanus toxoid IgG, but not with the T cell-independent IgG2 response to pneumococcal capsular polysaccaride. Our results indicate that measurement of organ and non-organ specific autoantibodies and IgG levels may be used to monitor disease activity in AIH.     

Organ and Non-organ Specific Autoantibody Titres and IgG Levels as Markers of Disease Activity: A Longitudinal Study in Childhood Autoimmune Liver Disease

No longitudinal study has investigated whether autoantibody titres and serum IgG levels correlate with disease activity in autoimmune liver disease. To determine this, we investigated prospectively 19 patients on 254 occasions between 10 months to 5 years from diagnosis. Nine had anti-nuclear and/or anti-smooth muscle antibody (ANA/SMA) positive autoimmune hepatitis (type 1 AIH), 5 liver kidney microsomal type 1 (LKM-1) positive AIH (type 2 AIH) and 5 ANA/SMA positive autoimmune sclerosing cholangitis (ASC). Correlation between IgG levels, titres of ANA, SMA and LKM-1 and levels of the organ specific autoantibodies anti-liver specific protein (anti-LSP), and anti-asialoglycoprotein receptor (anti-ASGPR) with biochemical evidence of disease activity, as measured by serum aspartate amino transferase (AST) levels, was sought during the course of the disease. AST levels correlated with levels of anti-LSP, anti-ASGPR and IgG in type 1 and 2 AIH, but not in ASC. Positive correlation with AST was also observed for LKM-1 titres in type 2 AIH and for SMA titres in type 1 AIH, but not in ASC. In both AIH and ASC, AST levels correlated with the T cell-dependent immune responses anti rubella IgG and anti tetanus toxoid IgG, but not with the T cell-independent IgG 2 response to pneumococcal capsular polysaccaride. Our results indicate that measurement of organ and non-organ specific autoantibodies and IgG levels may be used to monitor disease activity in AIH.     

IgG heterophile antibody causes false positivity for CA19-9, which is overcome with bovine immunoglobulin

Immunoassay using antibodies is widely applied to measure various clinical parameters such as tumor markers. However, many mechanisms of interference for this method have been reported. We studied serum sample showing false positive CA19-9 values in the range of 24 to approximately 286U/ml depending on the reagent lots used. PEG-pretreated serum did not show false positivity. Using high performance liquid chromatography (HPLC), the CA19-9 false positive peak was obtained around 160kD, which is the same as IgG. Antibody-coated microbeads, labeled antibody and buffer were used in different combinations between different lots, and the buffer was found to be the cause of false positive findings. The amount of bovine IgG contaminated in bovine serum albumin(BSA) in the reagent buffer markedly differed between lots of BSA, and reagent with a low amount of bovine IgG showed false positive results. Bovine immunoglobulin was superior to mouse IgG in the attempt to avoid a false positive reaction. We concluded that the cause of false positive CA19-9 findings in this serum sample was IgG heterophile antibody, which reacts with both of mouse and bovine IgG. The heterophile antibody had greater affinity to bovine IgG than to mouse IgG. The difference between CA19-9 values obtained by different reagent lots was due to different amounts of bovine IgG contaminated in BSA used in the reagent. Bovine immunoglobulin was superior to mouse IgG in the attempt to absorb the heterophile antibody and avoid false positive reaction in this case.     

吉林省供血者人类细小病毒B19 IgG抗体的调查

目的 了解吉林省供血者人类细小病毒感染的流行病学情况,为评估我国B19病毒的感染状况提供基础数据.方法 用间接ELISA方法检测血清中的抗B19 IgG抗体.结果 在184份血清中,抗B19 IgG抗体总检出率为55.43%.女性抗体阳性率高于男性,差异有统计学意义（P＜0.05）,年龄段在35～45岁之间的献血人员抗体阳性率最高.结论 本研究数据提示吉林省地区献血人员B19病毒感染率较高,有必要进行进一步B19 DNA的调查研究,为输血安全和血液制品安全提供保障.     

Recent progress of IgG4-related hepatobiliary diseases with emphasis on pathologic aspects and differential diagnosis

IgG4-related disease usually presents with a mass-forming or regional lesion showing considerable lymphoplasmacytic infiltration with many IgG4-positive plasma cells, sclerosis, and obliterative phlebitis. Herein, recent progress of hepatobiliary IgG4-related diseases including IgG4-related sclerosing cholangitis (IgG4-SC), hepatic inflammatory pseudotumor (HIP), and autoimmune hepatitis (AIH), are reviewed. IgG4-SC mainly affects the extrahepatic and hilar bile ducts, and is almost always associated with autoimmune pancreatitis (AIP), a prototype of IgG4-related disease. IgG4-SC has a propensity to exaggerate focally, and such IgG4-SC could be categorized as an IgG4-related HIP, mainly belonging to the lymphoplasmacytic type of HIP. IgG4-related AIH is a chronic active hepatitis with many IgG4-positive plasma cells and increased serum IgG4 level. In conclusion, several hepatobiliary diseases which had been diagnosed as other diseases, can be reclassified as IgG4-related diseases, and several local factors peculiar to the hepatobiliary system may be responsible for the development of these diseases.     

阴性少免疫沉积型新月体性肾炎患者血清AECA与临床表现的关系研究

目的：探讨抗中性粒细胞胞浆抗体（ANCA）阴性少免疫沉积型新月体性肾炎患者血清抗内皮细胞抗体（AECA）表达情况,及其与临床表现的关系。方法：选取2010年至2015年在我院治疗的少免疫沉积型新月体性肾小球肾炎患者,其中ANCA阴性患者45例（观察组）,ANCA阳性患者49例（对照组）,采用Western blot检测各组患者血清AECA水平。结果：观察组平均年龄和伯明翰血管炎活动性评分（BVAS）分别为（4108±943）岁和（1503±382）分,明显低于对照组（P＜005）;观察组出现发热、关节痛的比例分别为2667%和1333%,明显低于对照组（P＜005）;观察组出现肾病综合征比例为4889%,高于对照组（P＜005）;观察组血清AECA阳性率为4667%,明显低于对照组的8163%（P＜005）;观察组IgG AECA共识别7种蛋白,而对照组共识别11种蛋白,其中观察组抗90 kD抗体阳性率为1333%（6/45）,明显低于对照组的5102%（25/49）（P＜005）;观察组抗76 kD抗体阳性患者出现皮疹的比例为100%,明显高于阴性患者（P＜005）,抗200 kD抗体阳性患者BVAS评分为（1802±251）分,明显高于阴性患者（P＜005）。 〖HTH〗结论：〖HTSS〗ANCA阴性少免疫沉积型新月体性肾炎患者血清存在不同的AECA,可能与某些临床表现有关;ANCA阴性和阳性患者AECA有所不同,两者临床表现的差异可能与AECA的不同有关,需待进一步研究。     

Autoantibodies in Argentine women with recurrent pregnancy loss

PROBLEM: To determine the presence or absence of subclinical autoimmunity in Caucasian Argentine healthy women with first trimester recurrent pregnancy loss (RPL), the sera of 118 healthy women with a history of three or more consecutive abortions and 125 fertile control women without abortions and two children were analyzed for the presence of autoantibodies: immunoglobulin (Ig)G and IgM anticardiolipin, antinuclear (ANA), antismooth muscle (ASMA), antimitocondrial (AMA), antiliver-kidney-microsomal fraction (LKM), antigastric parietal cells (GPC), antineutrophil cytoplasmatic (ANCA) and antibodies antigliadin type IgA and IgG and IgA antitransglutaminase related with celiac disease (CD). METHOD OF STUDY: ANA, ASMA, AMA, anti-LKM, antibodies to GPC and ANCA were determined by indirect immunofluorescence (IFI) and anticardiolipin, antigliadina and antitransglutaminase antibodies were measured by enzyme-linked immunosorbent assays (ELISA). RESULTS: There was no significant difference between controls and patients with ANA, ASMA, AMA, LKM, ANCA and GPC. The prevalence of anticardiolipin antibodies in RPL was significantly higher than controls (P < 0,01) and the prevalence of positive antibodies for antigliadina type IgA and IgG and IgA antitransglutaminase in RPL was significantly higher than controls (P < 0.04). CONCLUSION: We show that Caucasian Argentine women with RPL showed significantly higher incidence of anticardiolipin antibodies than normal controls and finally we recommended the screening of IgA and IgG antigliadina and IgA antitransglutaminase antibodies in pregnancy, because of the high prevalence of subclinical CD in RPL and the chance of reversibility through consumption of a gluten free diet.     

丙基硫氧嘧啶引起抗中性粒细胞胞质抗体相关小血管炎并非药物性狼疮

目的 检测药物性狼疮（DIL）的血清学标志抗组蛋白抗体在抗中性粒细胞胞质抗体（ANCA）阳性的甲亢患者中的阳性率,并探讨丙基硫氧嘧啶（PITU）引起的ANCA相关小血管炎与DIL的关系。方法 以34例ANCA阳性的原发甲亢患者为研究对象,其中18例确诊为PITU引起的ANCA相关小血管炎;同时选取10例原发性ANCA相关小血管炎患者及10例初发未治疗的甲亢患者为对照,应用ELISA法检测抗组蛋白抗体。将我院确诊的PITU引起的ANCA相关小血管炎患者的临床资料与文献中抗甲状腺药物引起的DIL相比较。结果 所有原发性ANCA相关小血管炎的患者及初发未治疗的甲亢患者均未检测到抗组蛋白抗体;在34例PITU引起的ANCA阳性患者中仅1例（2．9％）抗组蛋白抗体阳性,此例患者为18例有血管炎临床表现中的1例（5．6％）。PITU引起的ANCA相关小血管炎不同于DIL,寡免疫复合物的坏死性新月体性肾炎及肺出血为前者的特征性表现。结论 PITU引起的ANCA相关小血管炎患者中抗组蛋白抗体的低发生率提示其与DIL可能不完全相同。     

Organ Correlation in IgG4-Related Diseases

IgG4-related disease (IgG4-RD) is a potentially multiorgan disorder. In this study, clinical and serological features from 132 IgG4-RD patients were compared about organ correlations. Underlying pathologies comprised autoimmune pancreatitis (AIP) in 85 cases, IgG4-related sclerosing cholangitis (IgG4-SC) in 12, IgG4-related sialadenitis (IgG4-SIA) in 56, IgG4-related dacryoadenitis (IgG4-DAC) in 38, IgG4-related lymphadenopathy (IgG4-LYM) in 20, IgG4-related retroperitoneal fibrosis (IgG4-RF) in 19, IgG4-related kidney disease (IgG4-KD) in 6, IgG4-related pseudotumor (IgG4-PT) in 3. Sixty-five patients (49%) had multiple IgG4-RD (two affected organs in 36 patients, three in 19, four in 8, five in 1, and six in 1). Serum IgG4 levels were significantly higher with multiple lesions than with a single lesion (P<0.001). The proportion of association with other IgG4-RD was 42% in AIP, the lowest of all IgG4-RDs. Serum IgG4 level was lower in AIP than in other IgG4-RDs. Frequently associated IgG4-RDs were SIA (25%) and DAC (12%) for AIP; AIP (75%) for IgG4-SC; DAC (57%), AIP (38%) and LYM (27%) for IgG4-SIA; AIP (26%) and LYM (26%) for IgG4-DAC; SIA (75%), DAC (50%) and AIP (45%) for IgG4-LYM; SIA (58%), AIP (42%) and LYM (32%) for IgG4-RF; AIP (100%) and SIA (67%) for IgG4-KID; and DAC (67%) and SIA (67%) for IgG4-PT. Most associated IgG4-RD lesions were diagnosed simultaneously, but IgG4-SIA and IgG4-DAC were sometimes identified before other lesions. About half of IgG4-RD patients had multiple IgG4-RD lesions, and some associations were seen between specific organs.

Graphical Abstract

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