IgC4相关肝胆疾病诊疗进展

IgG4相关性疾病是一类以淋巴浆细胞性炎症为主,伴血清和组织中IgG4增多并累及多器官或组织的慢性进行性全身性炎性疾病,累及肝胆胰腺系统时称之为IgG4相关肝胆疾病。简述了IgG4相关自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎的临床表现和实验室检查等方面。准确识别IgG4相关肝胆疾病的各个实验室结果,并正确诊断此类疾病,有助于避免不必要的手术和错误的治疗。     

IgG4相关肝胆疾病诊疗进展

IgG4相关性疾病是一类以淋巴浆细胞性炎症为主,伴血清和组织中IgG4增多并累及多器官或组织的慢性进行性全身性炎性疾病,累及肝胆胰腺系统时称之为IgG4相关肝胆疾病。简述了IgG4相关自身免疫性胰腺炎、IgG4相关硬化性胆管炎、IgG4相关自身免疫性肝炎的临床表现和实验室检查等方面。准确识别IgG4相关肝胆疾病的各个实验室结果,并正确诊断此类疾病,有助于避免不必要的手术和错误的治疗。     

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...     

CA19-9明显升高的IgG4相关性胆胰疾病1例报告

正随着近年来IgG4相关性疾病(immunoglobulin G4 relatedsystemic disease,IgG4-RD)概念的提出,该类疾病以组织中大量IgG4阳性浆细胞浸润、纤维化及闭塞性静脉炎为主要病理特征,可以累及多种器官或组织[1]。IgG4相关性胆胰疾病主要包括IgG4相关性自身免疫性胰腺炎、IgG4相关性硬化性胆管炎等。部分IgG4相关性胆胰疾病患者可见CA19-9升高,但     

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相关肝胆胰疾病发病机制尚未完全明确, 现重点报道IgG4相关肝胆胰疾病近年来在肠道微生态-免疫学、宿主遗传-免疫及复发监测领域的进展。     

以阻塞性通气功能障碍为首发表现的IgG4相关性疾病1例并文献复习

<正>IgG4相关性疾病(immunoglobulin G4-related disease, IgG4-RD)是一组由免疫介导的慢性自身炎症性疾病，可累及全身多个器官及组织；日本学者在 2003 年首次提出了 IgG4 相关性肺疾病(IgG4-related lung disease, IgG4-RLD)的概念，指发生在呼吸系统、胸膜、纵隔的IgG4-RD,临床表现可与多种疾病相似，易漏诊、误诊^[1]。     

IgG4相关性消化疾病及治疗研究进展

IgG4相关性疾病(IgG4-RD)是一组病因不明的表现为慢性纤维化的系统性自身免疫疾病,可波及全身器官,以IgG4升高以及IgG4阳性浆细胞浸润受累组织器官为特征,在消化系统中主要累及胰腺、肝脏、胆道、腹膜后等,糖皮质激素是目前公认的治疗IgG4-RD的一线用药,几乎所有的患者对于糖皮质激素治疗反应良好.     

以自身免疫性胰腺炎和Mikulicz病为特征且伴有白细胞降低的IgG4相关性疾病1例报告

Ig G4相关性疾病(IgG4-related disease)是一类慢性、进行性炎症伴有纤维化的自身免疫性疾病,特征性的表现为血清IgG4水平升高,组织中大量IgG4阳性浆细胞浸润,席纹状纤维化以及闭塞性静脉炎[1-2],临床上病变几乎可以累及所有的器官和器官[3],受累器官通常表现为弥漫性的肿大。在消化系统中,唾液腺和胰腺是最常累及的器官。现将我们收治的以自身免疫性胰腺炎和Mikulicz病为特征且伴有白细胞降低的IgG4相关性疾病1例报告如下。     

IgG4相关性疾病

IgG4相关性疾病是一组以血清IgG4水平升高、受累组织IgG4阳性浆细胞浸润及纤维化为特征的、近几年才被人们认识的新疾病体.受累器官因纤维化、慢性炎症等出现增生肿大,从而导致相应的压迫阻塞症状或功能障碍.该病激素治疗效果较好[1-2].该病自发现至今有不少于10种名称,如IgG4相关的硬化性疾病、IgG4相关的自身免疫病、高IgG4疾病、IgG4阳性多器官淋巴增殖综合征(IgG4-positive multiorgan lymphoproliferative syndrome,IgG4+MOLPS)等[3],直到2010年才被统一命名为IgG4相关性疾病[4].本研究将从该病的认识过程、临床特点、病理特点、发病机制、诊断、治疗等方面作一综述,以便大家能逐渐认识和掌握该病.     

同时累及胸主动脉腹主动脉及颌下腺的IgG4相关性疾病1例报告并文献复习北大核心CSCD

IgG4相关性疾病是一种炎性-纤维化疾病,通常以单个或多个器官肿大起病,组织中IgG4＋浆细胞增多,伴或不伴血清中IgG4升高。常见受累器官包括泪腺、颌下腺、甲状腺、肺、肾脏、胰腺、胆管、淋巴结、腹膜后间隙等,累及的器官或组织由于炎性一纤维病变继发弥漫性肿大,除受累器官本身的表现外,尚有其压迫临近器官或组织而引起的一系列临床表现。现将本文收治的1例同时累及胸主腹主动脉及颌下腺的IgG4相关性疾病报道如下。     

Infrequent organ involvement of IgG4-related diseases: a literature review

IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD.     

IgG4相关性疾病诊治中国专家共识

IgG4相关性疾病(IgG4-RD)是一种较罕见的由免疫介导的慢性炎症伴纤维化疾病,可累及全身多个器官和系统,临床表现复杂多样。由于对该病认识时间较短,我国IgG4-RD的整体诊治水平参差不齐,国内亦无相关专家共识或诊治指南。为进一步提高各专业医生对IgG4-RD的认识和规范诊治水平,由中国罕见病联盟与中华医学会风湿病学分会联合组织专家组,在总结国内外经验和研究结果的基础上,制定了本共识,旨在统一我国临床医生对该病的诊治认识,减少漏诊和误诊,改善患者预后。     

Riedel's Thyroiditis and Multifocal Fibrosclerosis are part of the IgG4‐related systemic disease spectrum

Objective

Riedel's thyroiditis is a chronic fibrosing disorder of unknown etiology often associated with “multifocal fibrosclerosis.” IgG4‐related systemic disease is characterized by IgG4+ plasma cell infiltration and fibrosis throughout many organs. We hypothesized that Riedel's thyroiditis is part of the IgG4‐related systemic disease spectrum.

Methods

We searched our institution's pathology database using the terms “Riedel's,” “struma,” “thyroid,” and “fibrosis,” and identified 3 cases of Riedel's thyroiditis. Riedel's thyroiditis was diagnosed if there was a fibroinflammatory process involving all or a portion of the thyroid gland, with evidence of extension of the process into surrounding tissues. Immunohistochemical stains for IgG4 and IgG were performed. The histopathologic and immunohistochemical features of each involved organ were evaluated. The clinical features of one patient with multiple organ system disease were described.

Results

All 3 thyroidectomy samples stained positively for IgG4‐bearing plasma cells. One patient had extensive extrathyroidal involvement diagnostic of IgG4‐related systemic disease, including cholangitis, pseudotumors of both the lung and lacrimal gland, and a lymph node contiguous to the thyroid that stained intensely for IgG4+ plasma cells. The histologic features of all organs involved were consistent with IgG4‐related systemic disease. Patient 3 had 10 IgG4+ plasma cells per high‐power field initially, but rebiopsy 2 years later demonstrated no IgG4+ plasma cells. That patient's second biopsy, characterized by fibrosis and minimal residual inflammation, further solidifies the link between IgG4‐bearing plasma cells in tissue and the histologic evolution to Riedel's thyroiditis.

Conclusion

Riedel's thyroiditis is part of the IgG4‐related systemic disease spectrum. In many cases, multifocal fibrosclerosis and IgG4‐related systemic disease are probably the same entity.     

Risk of malignancies in IgG4-related disease

Abstract

IgG4-related disease (IgG4-RD) is considered a systemic, chronic, and inflammatory disorder that is characterized by the enlargement of involved organs, elevated levels of IgG4, and abundant infiltration of plasmacytes with IgG4 and fibrosis in involved organs. It is necessary to differentiate IgG4-RD from malignant tumors. Recently we have looked at case reports of IgG4-RD with malignancy that was discovered at systemic screening. In this study, we analyzed the relationship between IgG4-RD and malignancies. The study subjects were 106 patients with IgG4-RD who had been referred to our hospital since April 1997. We analyzed the clinical characteristics of IgG4-RD patients who had cancer that was observed upon the initial diagnosis of IgG4-RD or that occurred during an average follow-up period of 3.1 years. Using data from national cancer registries that monitor cancer incidence in Japan, we evaluated the standardized incidence ratio (SIR) for malignancies in IgG4-RD. Malignancies were observed in 11 of the IgG4-RD patients (10.4%). The malignancies were all different and included lung cancer, colon cancer, and lymphoma. With the exception of the age at which the IgG4-RD diagnosis was made, there were no common features in patients with cancer and those without. The SIR for these malignancies in IgG4-RD was 383.0, which was higher than that for the general population. We should be cognizant of the possible existence of malignancies in patients with IgG4-RD at the time of diagnosis and during follow-up care.     

Risk of malignancies in IgG4-related disease

IgG4-related disease (IgG4-RD) is considered a systemic, chronic, and inflammatory disorder that is characterized by the enlargement of involved organs, elevated levels of IgG4, and abundant infiltration of plasmacytes with IgG4 and fibrosis in involved organs. It is necessary to differentiate IgG4-RD from malignant tumors. Recently we have looked at case reports of IgG4-RD with malignancy that was discovered at systemic screening. In this study, we analyzed the relationship between IgG4-RD and malignancies. The study subjects were 106 patients with IgG4-RD who had been referred to our hospital since April 1997. We analyzed the clinical characteristics of IgG4-RD patients who had cancer that was observed upon the initial diagnosis of IgG4-RD or that occurred during an average follow-up period of 3.1 years. Using data from national cancer registries that monitor cancer incidence in Japan, we evaluated the standardized incidence ratio (SIR) for malignancies in IgG4-RD. Malignancies were observed in 11 of the IgG4-RD patients (10.4%). The malignancies were all different and included lung cancer, colon cancer, and lymphoma. With the exception of the age at which the IgG4-RD diagnosis was made, there were no common features in patients with cancer and those without. The SIR for these malignancies in IgG4-RD was 383.0, which was higher than that for the general population. We should be cognizant of the possible existence of malignancies in patients with IgG4-RD at the time of diagnosis and during follow-up care.     

Peripheral T-cell lymphoma that developed during the follow-up of IgG4-related disease

IgG4-related disease is a recently recognized fibroinflammatory disorder characterized by extensive IgG4-positive plasma cell and lymphocyte infiltration of various organs. The pancreatic manifestation of IgG4-related disease is called autoimmune pancreatitis (AIP), in which autoimmune mechanisms are likely involved. On the other hand, some autoimmune and chronic inflammatory disorders, such as Sj?gren's syndrome and rheumatoid arthritis, are associated with increased risks of non-Hodgkin lymphoma (NHL). There have been a few reports of cases with IgG4-related disease that had subsequently developed NHL, however, all of them suffered from B-cell lymphoma. We describe the first case of NHL, compatible with a subtype of peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), which arose in IgG4-related sclerosing cholangitis. As patients with IgG4-related disease may be at an increased risk of developing NHL, such presentation during the follow-up of IgG4-related disease should be carefully scrutinized to exclude NHL.     

Sclerosing Cholangitis Related to IgG4: Not Always a Curable Entity

IgG4-related disease is a recently-described fibro-inflammatory condition with characteristic histopathological findings in the organs involved. The most commonly affected organs are pancreas, lymph nodes, and retroperitoneum. Liver disease usually involves bile structures and therefore IgG4-related disease is considered a cause of secondary sclerosing cholangitis. One out of three patients with IgG4 sclerosing cholangitis also presents autoimmune pancreatitis, although it can be associated with manifestations in other organs. One of the main features of IgG4-related disease is its good prognosis due to the great response to glucocorticoid therapy. However, relapse of the disease is not uncommon, especially when steroid therapy is decreased or stopped. Rituximab seems to be an effective treatment to achieve remission of the disease. We report the case of a 74 year-old man diagnosed with IgG4-related disease based on increase of serum IgG4 levels, imaging and histopathological findings, with systemic involvement including sclerosing cholangitis. Despite the absence of liver fibrosis at onset, the early use of glucocorticoids and rituximab therapy, the patient presented clinical and analytical deterioration, leading to secondary biliary cirrhosis. In conclusion, this clinical case highlights the importance of prompt diagnosis and therapeutics for sclerosing cholangitis secondary to IgG4-related disease in order to avoid progression of the disease and development of liver cirrhosis, as well as the refractory, aggressive nature of the disease in some cases as this one.     

Immunoglobulin G4 (IgG4)-related autoimmune hepatitis and IgG4-hepatopathy: A histopathological and clinical perspective

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic inflammatory disease that simultaneously or consecutively involves multiple organs of the body. It is characterized by elevated serum IgG4 levels and massive infiltration of IgG4⁺ plasma cells in the damaged tissues. IgG4-related autoimmune hepatitis (IgG4-AIH) and IgG4-hepatopathy are relatively new entities that have been proposed as a phenotype of IgG4-RD in the liver. Immunoglobulin G4-AIH is defined as a disorder with serological, histopathological, and clinical features of both IgG4-RD and AIH, simultaneously satisfying the diagnostic criteria of both classical AIH and IgG4-RD. Although there are several case reports and studies of IgG4-AIH among the published works, no consensus regarding the histopathological characteristics of IgG4-AIH has been established, and its clinical implications remain obscure. Immunoglobulin G4-hepatopathy is defined as a comorbidity of IgG4-RD in the liver, and patients not meeting the diagnostic criteria of classical AIH could be diagnosed with IgG4-hepatopathy. Numerous issues regarding these diseases, especially their epidemiology, histopathological and clinical characteristics, and treatment response to corticosteroids, remain unsolved, and need to be determined to establish the disease concepts of IgG4-AIH and IgG4-hepathopathy.     

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相关系统性疾病的肾脏损害

IgG4相关系统性疾病(IgG4-related systemic disease,IgG4-RSD)是一组最近认识的临床综合征,可累及多个器官,胰腺最常受累,主要表现为血清IgG4升高,受累组织弥漫IgG4阳性浆细胞浸润。本文主要介绍IgG4-RSD累及肾脏时的临床表现、影像学、实验室检查、病理特点及治疗情况。