IgG4-Related Disease: A Constellation of Abdominal Manifestations

IgG4-related disease is an autoimmune condition that results in inflammation and fibrosis throughout multiple organ systems. This disease is rare; however, it can mimic many more prevalent conditions clinically and thus should be included in their differential diagnoses. Although autoimmune pancreatitis is the most common abdominal manifestation, the disease can afflict the hepatobiliary, vascular and renal systems as well. We present a case of a 78-year-old male with symptoms of chronic fatigue and weight loss. Imaging was performed with the expectation of revealing a malignancy, although the features of IgG4-related disease involving multiple organs were detected instead. Serology confirmed the diagnosis. It is imperative to diagnose IgG4-related disease early as it has a different treatment and favorable prognosis compared to many malignancies.     

小鼠IgG抗体的制备——IgG—SPA菌体免疫法

本文介绍制备小鼠IgG抗体的一种简便方法,即IgG-SPA菌体免疫法。首先制备10％(w/v)Cowan I 株菌体稳定液,按一定比例与正常小鼠血清制备IgG-SPA菌体免疫原。然后以9—12亿/ml菌体免疫原静脉注射于年青健康家兔,数次免疫后,即得兔抗小鼠IgG抗体(RAM-IgG),其血清效价达1:32～1:64(双扩法);其抗体纯度高,与正常小鼠血清只出现一条沉淀线。经鉴定,其血清不含有鼠IgM抗体。本文又把该法与IgG常规乳化佐剂免疫法作了比较。     

难治性溃疡性结肠炎患者术前食物不耐受情况及相关因素分析

目的：综合分析难治性溃疡性结肠炎患者食物不耐受情况,为指定个体化饮食方案提供依据。方法采用美国BIO-MERICA公司生产的食物过敏原检测试剂盒,用酶联免疫方法（ELISA）检测血清中以14种食物为过敏原产生的特异性免疫球蛋白G（IgG）抗体水平,对获得资料进行综合分析。结果42例难治性溃疡性结肠炎患者食物不耐受阳性率为78.57％（33／42）,排在前三位的不耐受食物依次为：牛奶／羊奶（35.71％）,蛋清／蛋黄（28.57％）,蟹（23.81％）。结论难治性溃疡性结肠炎患者食物不耐受发生率较高,且与性别、年龄、营养等多种因素有关,在为患者制定饮食方案时应个体化综合考虑。     

Clinical and histological changes associated with corticosteroid therapy in IgG4-related tubulointerstitial nephritis

Abstract

Objectives This study aimed to investigate the clinicopathological changes induced by corticosteroid therapy in immunoglobulin (Ig)G4-related tubulointerstitial nephritis (TIN).

Methods We studied six IgG4-related TIN patients receiving renal biopsies before and after corticosteroid therapy. Their clinical data and histological findings were evaluated before and after therapy.

Results Elevated serum creatinine levels rapidly improved after corticosteroid therapy except for two patients, in whom it persisted. Abnormal radiological findings improved in all patients, although focal cortical atrophy persisted in three. Histologically, TIN-like dense lymphoplasmacytic infiltration, interstitial fibrosis, IgG4-positive plasma cell, CD4+CD25+ T cell, and Foxp3+ cell infiltration were characteristic before therapy. After therapy, the area with cell infiltration decreased and regional fibrosis became evident in the renal interstitium. The number of IgG4-positive plasma cells and Foxp3+ cells significantly diminished even in the early stage of therapy, whereas low to moderate numbers of CD4+ and CD8+ T cells still infiltrated where inflammation persisted in the later stage.

Conclusions Our study shows that persistent renal insufficiency associated with macroscopic atrophy and microscopic fibrosis is not so rare in IgG4-related TIN. Pathologically, the behavior of regulatory T cells during the clinical course is quite similar to that of IgG4-positive plasma cells, and the behavior pattern of those cells is distinctive.     

Performance evaluation of serum IgG subclass quantification using a SPAPLUS turbidimetric analyzer and comparison with the BNII nephelometer

Abstract

IgG consists of four subclasses: IgG1, IgG2, IgG3, and IgG4. Changes in the serum concentration of each subclass reflect different clinical situations, and quantification of each subclass is important to assess patients’ clinical states. Herein, we evaluated the analytical performance of the SPAPLUS turbidimetric analyzer (The Binding Site, Birmingham, UK) for IgG subclass. Precision, linearity, comparison with the BNII system (Siemens Healthineers, Erlangen, Germany), and reference interval were assessed according to the Clinical and Laboratory Standards Institute (CLSI) guidelines. The repeatability and within-laboratory precision were within 5% for all IgG subclasses. The coefficient of determination (R²) was higher than 0.99 for the analytical measurement range in all IgG subclasses. Comparison between SPAPLUS and BNII revealed significant differences in IgG1, IgG3, and IgG4 (p?相似文献   

脑脊液白蛋白和免疫球蛋白IgG检测在儿童病毒性脑炎诊治中的应用

目的：探讨病毒性脑炎患儿脑脊液白蛋白比值（QAlb)、IgG和IgG指数变化与疾病严重程度的相关性。方法研究对象分病毒性脑炎组和对照组,病毒性脑炎组按临床特点分为轻症病毒性脑炎组和重症病毒性脑炎组,采用免疫散射比浊法测定血清和脑脊液的白蛋白、IgG,计算QAlb和IgG指数,判断病毒性脑炎患儿血脑屏障功能状态及免疫球蛋白鞘内合成情况与临床严重程度的关系。结果病毒性脑炎组的QAlb高于对照组;重症病毒性脑炎组的QAlb较轻症病毒性脑炎组和对照组增高,差异有统计学意义（P<0.05）。重症病毒性脑炎组脑脊液IgG、IgG指数比轻症病毒性脑炎组和对照组增高,差异有统计学意义（P<0.05）。结论重症病毒性脑炎组患儿的血脑屏障功能损害程度、鞘内IgG合成情况与临床病情严重程度相关。检测脑脊液和血清中白蛋白、IgG,计算QAlb和IgG指数能反映病毒性脑炎患儿血脑屏障功能状态及IgG鞘内合成情况,为判断病毒性脑炎临床病情的严重程度提供相关依据。     

基于IgG抗A（B）效价梯度及动态差值探讨ABO-HDN病情相关性

目的探讨动态监测IgG抗A(B)效价梯度与ABO-HDN病情严重程度临床指标的相关性。方法选取2017年1~5月间47例ABO溶血病患儿临床资料进行统计分析,记录患儿出生后32 h的血清总胆红素(serum total bilirubin,STB_(32h))、血红蛋白(hemoglobin,Hb)、网织红细胞百分比(the percentage of reticulocyte,Ret%)及孕妇第16和37周IgG抗A(B)效价梯度。比较各组新生儿临床指标和lgG抗A(B)效价的相关性。结果 STB_(32h)与37周IgG抗A(B)效价梯度、IgG抗A(B)_(37-16)梯度差呈正相关(P0.001);Hb与第37周IgG抗A(B)效价梯度呈负相关(r~2=0.2074,P=0.0013);Hb与IgG抗A(B)_(37-16)梯度差值呈负相关(r~2=0.1831;P=0.0027);Ret%与37周IgG抗A(B)效价梯度、IgG抗A(B)_(37-16)梯度差值呈正相关(P0.001)。结论产前37周IgG抗A(B)效价梯度、IgG抗A(B)_(37-16)效价梯度差值与新生儿ABO-HDN的病情严重程度具有一定相关性。     

IgG4-related disease

The term “IgG4-related disease” encompasses several disorders described many years ago under various designations depending on the organ or system involved (e.g., Mikulicz syndrome, Riedel's thyroiditis, and retroperitoneal fibrosis). The clinical presentation varies widely, as one or more organs may be affected, usually in the same region of the body and either synchronously or metachronously. The main targets are the pancreas, bile ducts, salivary glands, lachrymal glands, mediastinal lymph nodes, and retroperitoneum. IgG4-related disease is rare, with an estimated incidence of 0.2 to 1/100 000 in Japan and no available incidence data in occidental countries. Men older than 50 years are predominantly affected. Serum IgG4 levels are often greater than 1.35 g/L. Enlargement of the involved organs, which may be pseudotumoral, is due to a combination of infiltration by T cells and IgG4-expressing plasma cells, storiform fibrosis, and obliterative thrombophlebitis. Glucocorticoid therapy is effective but may be followed by relapses requiring the use of immunomodulating agents such as azathioprine, methotrexate and, more recently, rituximab. IgG4-related disease is not an autoimmune condition related to IgG4 autoantibodies, and neither does it involve immune complexes. Specific joint involvement has been reported in a very small number of patients.     

睾丸性无精子症中医证型间睾丸病理类型、IgG、IgM的比较研究

目的 通过对睾丸性无精子症肾虚证组、血瘀证组患者睾丸病理类型、IgG、IgM、的比较研究，为辨证论治睾丸性无精子症提供理论依据。方法 31例睾丸性无精子症患者的睾丸组织，其中。肾虚证者16例，血瘀证者15例。正常对照组睾丸5例。进行病理检查，睾丸病变程度行Makler评分，利用免疫组织化学ABC法检测睾丸组织中IgG、IgM沉积情况，并对上述结果行统计学处理。结果两组患者的病理类型都以生精阻滞为主，但血瘀证组患者的睾丸病变更为严重而复杂。31例患者中22例有IgG沉积，11例有IgM沉积，两组间比较无显著性差异，免疫复合物的沉积与睾丸病变程度无关。结论血瘀证患者睾丸组织病变更为严重。自身免疫在肾虚证、血瘀证患者中普遍存在，但免疫因素在睾丸性无精症的发病机制中不占主导地位，自身免疫与证型无关。