免疫球蛋白G4相关性冠状动脉血管炎一例

正1临床资料患者男性,62岁,主因心慌、胸闷10余天,于2019年8月7日入我院就诊。冠状动脉CT血管成像(CCTA)检查见图1～8。图1～8显示:冠状动脉左主干、前降支、右冠状动脉钙化及非钙化斑块,管腔中～重度狭窄,左主干、前降支、回旋支、右冠状动脉节段性管壁增厚,管腔周围软组织密度影环绕,     

免疫球蛋白G4相关性胆管炎诊断和治疗进展

一、概述1995年,日本学者报道了一种以血清免疫球蛋白(Immunoglobulin,IG) G4水平升高和IgG4阳性淋巴-浆细胞的组织浸润为特征的纤维炎症性疾病,即自身免疫性胰腺炎(autoimmune pancreatitis,AIP)[1].现已认识到AIP是一世界性疾病.其特征性表现包括胰腺肿大或肿块(可能类似于恶性肿瘤)、血清IgG4水平升高、组织淋巴-浆细胞浸润和激素治疗有效[2].     

免疫球蛋白G4相关肝胆胰疾病的微生态免疫学进展

免疫球蛋白（Ig）G4相关疾病是一种免疫介导的累及多个器官的慢性纤维炎性疾病。IgG4相关肝胆胰疾病是IgG4相关疾病在肝胆胰系统中的表现,以血清IgG4滴度升高、病变组织中大量IgG4阳性浆细胞浸润、组织纤维化、影像学器官形态改变等为主要特点,因缺乏可靠的生物标志物,组织病理学仍是诊断的重要依据。IgG4相关肝胆胰疾...     

血清免疫球蛋白(Ig)G4在非IgG4相关性肝胆疾病中的表达

回顾性分析因腹痛、黄疸、肝功能异常等肝胆系统相关症状而就诊并进行血清免疫球蛋白(Ig)G4检测的患者,探讨血清IgG4在非IgG4相关性肝胆疾病患者中的表达情况及其临床意义,发现血清IgG4水平升高亦见于非IgG4相关性肝胆疾病(IgG4-RD)患者,性别和年龄可能对血清IgG4水平造成一定的影响。通过随访IgG4-RD患者的血清IgG4水平和/或病理组织学检查有助于提高对IgG4-RD以及血清IgG4水平价值的认识。     

免疫球蛋白G4相关性疾病与恶性肿瘤

免疫球蛋白G4(immunoglobulin G4,IgG4)相关性疾病是一组具有特异性IgG4阳性浆细胞浸润表现的炎症性疾病。近年来对于IgG4相关性疾病并发恶性肿瘤的报道逐渐增多,主要有淋巴瘤和其他非淋巴性肿瘤。现阶段研究认为IgG4相关性疾病患者发生恶性肿瘤的风险增加,不同部位的IgG4相关性疾病诱导发生的恶性肿瘤类型具有一定的倾向性,而恶性肿瘤可能与IgG4相关性疾病的发生有关。IgG4的生物学特性和功能与IgG4相关性疾病的组织炎症表现可能帮助解释两种疾病之间的关系和发生机制。     

免疫球蛋白G4相关性疾病冠状动脉炎性假瘤1例

1 病例资料 患者,男性,58岁,2006年因"间断腹痛8年,体重下降2个月"入院,表现为劳累后出现上腹部胀满和疼痛不适,伴纳差、反复口腔溃疡、双眼睑肿胀.入院后腹部超声提示胰腺体尾部直径3.0 cm占位性病变.PET-CT:胰腺体尾部萎缩,胰头及钩突部饱满,未见明确异常代谢增高改变.血清IgG4测定 401.0 mg...     

IgG4相关性消化疾病的研究进展

IgCA相关性疾病是一种新认识的纤维炎性疾病,其特征是肿块样病灶、以IgG4阳性浆细胞为主的致密的淋巴浆细胞的浸润、受累组织呈轮辐状纤维化,通常伴有血清IgG4升高。自身免疫性胰腺炎（autoimmune pancreatitis,AIP）被认为是一种IgG4相关性系统性疾病,最早是由Yoshida等在1995年提出,     

免疫球蛋白G4相关性自身免疫性胰腺炎合并肝损害1例报告

1病例资料患者女性,57岁,汉族,天津籍。因＂纳差伴恶心2月余＂于2012年2月3日入院。患者于2余月前无明显诱因出现食欲减退、恶心,食量较前未明显减少。既往于2010年3月发现自身免疫性胰腺炎（autoimmune pancreatitis,AIP）,未用激素治疗,随后发现血糖升高,先后服用格列美脲、拜糖平治疗,平素空腹血糖控制在8～9 mmol/L。     

IgG4相关间质性肺疾病

IgG4相关间质性肺疾病的提出是基于对系统性IgG4相关硬化性疾病的研究发现.IgG4相关间质性肺疾病的诊断需要临床医师结合临床-影像学-病理学特征疑及此病.IgG4相关间质性肺疾病对糖皮质激素治疗反应良好,监测随访对于发现复发是必要的.     

Recent advances in the concept and diagnosis of autoimmune pancreatitis and IgG4-related disease

Recent studies have suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 AIP, related to IgG4 (lymphoplasmacytic sclerosing pancreatitis); and type 2 AIP, related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis). Compared with type 2 AIP, the clinicopathological features of type 1 AIP, with increased serum IgG4/IgE levels, abundant infiltration of IgG4 + plasmacytes and lymphocytes, autoantibodies, and steroid responsiveness, are more suggestive of abnormal immunity such as allergy or autoimmunity. Moreover, patients with type 1 AIP often have extrapancreatic lesions, such as sclerosing cholangitis, sclerosing sialadenitis, or retroperitoneal fibrosis, showing pathological features similar to those of the pancreatic lesions. Based on these findings, an international concept of and diagnostic criteria for AIP have been proposed recently. Of interest, many synonyms have been proposed for the conditions of AIP and extrapancreatic lesions associated with IgG4, such as "multifocal idiopathic fibrosclerosis," "IgG4-related autoimmune disease," "IgG4-related sclerosing disease," "systemic IgG4-related plasmacytic syndrome (SIPS)," and "IgG4-related multiorgan lymphoproliferative syndrome," all of which may refer to the same conditions. Therefore, the Japanese Research Committee for "Systemic IgG4-Related Sclerosing Disease" proposed a disease concept and clinical diagnostic criteria based on the concept of multifocal fibrosclerosing disease, in 2009, in which the term "IgG4-related disease" was agreed upon as a minimal consensus to cover these conditions. Although the significance of IgG4 in the development of "IgG4-related disease" remains unclear, we have proposed a hypothesis for the development of type 1 AIP, one of the IgG4-related diseases. The concept and diagnostic criteria of "IgG4-related disease" will be changed in accordance with future studies.     

IgG4-related respiratory disease

Abstract

IgG4-related diseases (IgG4-RDs), such as autoimmune pancreatitis and IgG4-related Mikulicz disease, are often accompanied by intrathoracic lesions, which are called IgG4-related respiratory disease (IgG4-RRD). IgG4-RRD has few subjective symptoms, and is usually detected during workup of patients with extra-thoracic lesions of IgG4-RD. IgG4-RRD is characterized by various conditions, including masses, nodules, thickening, and infiltration at numerous sites in the thorax through lymphatic routes. Although elevated serum IgG4 concentrations and pathologic evidence of lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells are characteristic findings of IgG4-RD, other intrathoracic diseases, such as multicentric Castleman disease and malignancy, may present with similar findings. Developing diagnostic criteria for IgG4-RRD, including clinicoradiological and pathological characteristics, is necessary for its appropriate diagnosis.     

IgG4-related sclerosing disease

Based on histological and immunohistochemical exami- nation of various organs of patients with autoimmune pancreatitis （AIP）, a novel clinicopathological entity of IgG4-related sclerosing disease has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration of various organs. Clinical manifestations are apparent in the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prosrate, in which tissue fibrosis with obliterative phlebitis is pathologically induced. AlP is not simply pancreatitis but, in fact, is a pancreatic disease indicative of IgG4- related sclerosing diseases. This disease includes AlP, sclerosing cholangitis, cholecystitis, sialadenitis, retro-peritoneal fibrosis, tubulointerstitial nephritis, interstitial pneumonia, prostatitis, inflammatory pseudotumor and lymphadenopathy, all IgG4-related. Most IgG4-related sclerosing diseases have been found to be associated with AlP, but also those without pancreatic involvement have been reported. In some cases, only one or two organs are clinically involved, while in others, three or four organs are affected. The disease occurs predominantly in older men and responds well to steroid therapy. Serum IgG4 levels and immunos-taining with anti-IgG4 antibody are useful in making the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.     

IgG4相关性肺疾病1例及文献复习

目的提高对IgG4相关性肺疾病的临床特征、胸部影像学和病理组织学的认识。方法对1例经病理证实的IgG4相关性肺疾病的临床资料进行分析,并结合文献进行回顾总结。结果患者男,62岁,以胸腔积液起病,在外院先后行胸膜活检、胸腔镜肺活检等均未能明确诊断,后至我院行CT引导下经皮肺穿刺活检术,结果示活检组织内纤维组织增生伴淋巴细胞、浆细胞等炎症细胞浸润,免疫组化示大量浆细胞(+),IgG4阳性,浆细胞最密集计数约为40个/高倍视野,血清IgG4浓度示4.07 g/L(0.03 g/L~2 g/L)。诊断IgG4相关性肺疾病,给予糖皮质激素治疗,2个月后复查胸部CT示肺部病灶较前局部吸收、好转。结论IgG4相关性疾病是一种累及多器官、以血清IgG4水平升高、组织IgG4阳性浆细胞浸润为特点的淋巴浆细胞病。目前国内IgG4相关性肺疾病的报道很少,报道这一病例并进行文献复习有助于提高对IgG4相关性肺疾病的认识。     

Treatment of IgG4-related disease

IgG4-related disease (IgG4-RD) is capable of causing great morbidity and even mortality if the condition remains undiagnosed or poorly treated, yet is typically a treatment-responsive disorder. Glucocorticoids have not been studied rigorously and practices with regard to dosing and duration of treatment remain largely empiric. In addition, IgG4-RD patients are often particularly susceptible to and intolerant of the deleterious effects of glucocorticoid therapy. B cell depletion with anti-CD20 monoclonal antibodies appears to be a rapid, effective means of obtaining disease control and limiting patients’ glucocorticoid exposure, but this option is frequently not available. Other therapies targeting the B cell lineage may also be efficacious, and one is under study. The means by which depletion or inhibition of B cells and their progeny ameliorate IgG4-RD is coming into focus now through careful mechanistic studies of samples from treated patients. The mechanistic understanding of IgG4-RD will bring an array of specific targets for therapeutic intervention. Plasmablast-directed therapy with a CD19 monoclonal antibody is currently in clinical trials. CD4?+ cytotoxic T lymphocytes and fibrosis, both observed nearly universally in the tissue of IgG4-RD patients, present two unexploited vulnerabilities in controlling and even reversing the effects of the disease. Further development of such therapies is a major goal of the next few years.