Masaaki Nagae, Joji Onishi, Hiroyuki Umegaki, Masafumi Kuzuya
IgG4-related disease (IgG4-RD) which is characterized by an autoimmune abnormality and elevated serum IgG4 level often presents as swelling, nodules, and the thickening of multiple organs. It typically occurs in middle-aged to elderly patients, but its pathogenesis remains unclear. Lymphadenopathy is frequently seen in IgG4-RD, which clinically requires a differential diagnosis from malignant lymphoma. We herein report a case of malignant lymphoma with IgG4-RD arising in a very elderly man.An 85-year old man with a 6-year history of autoimmune pancreatitis was diagnosed as IgG4-related hypophysitis 13 months prior to admission...
2020: Nihon Ronen Igakkai Zasshi. Japanese Journal of Geriatrics
Jacob R Bledsoe, Judith A Ferry, Azfar Neyaz, Leonardo Boiocchi, Cara Strock, Karen Dresser, Lawrence Zukerberg, Vikram Deshpande
Lymphadenopathy is common in patients with immunoglobulin G4-related disease (IgG4-RD). However, the described histopathologic features of IgG4-related lymphadenopathy have been shown to be largely nonspecific. In an attempt to identify features specific for nodal IgG4-RD we examined the histopathologic features of lymph nodes from 41 patients with established IgG4-RD, with comparison to 60 lymph nodes from patients without known or subsequent development of IgG4-RD. An increase in immunoglobulin (Ig) G4-positive plasma cells >100/HPF and IgG4/IgG ratio >40% was identified in 51% of IgG4-RD cases and 20% of control cases...
September 1, 2020: American Journal of Surgical Pathology
Manxuan Pend, Lizhen Yang, Shangjie Wu
IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosis disease characterized by elevated serum IgG4 and tissues as well as organs infiltrated with IgG4-positive cells, resulting in swelling and damage.It is currently treated as first-line treatment with glucocorticoids. Autoimmune hemolytic anemia (AIHA) is also a relatively rare disease that caused by autoreactive erythrocyte antibodies. Although both are autoimmune-related diseases, they rarely overlap. The relationship between them is not clear. A case of IgG4-RD combined with AIHA is reported...
June 28, 2020: Zhong Nan da Xue Xue Bao. Yi Xue Ban, Journal of Central South University. Medical Sciences
Sandra Strainiene, Lukas Sarlauskas, Ilona Savlan, Valentina Liakina, Ieva Stundiene, Jonas Valantinas
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease that affects almost every organ and is a diagnostic challenge for every clinician. There is a lack of adequate epidemiological data worldwide, and evidence-based treatment recommendations are not yet established. We report the first case of IgG4-RD from Lithuania and the Baltic Sea region presented with thyroiditis, orbital myositis, orbitopathy, uveitis, scleritis, sialadenitis, autoimmune pancreatitis and prostatitis...
August 6, 2020: World Journal of Clinical Cases
Ji Woon Oh, Sung Eun Rha, Moon Hyung Choi, Soon Nam Oh, Seo Yeon Youn, Joon-Il Choi
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by focal or diffuse organ infiltration of IgG4-bearing plasma cells. The diagnosis of IgG4-RD is based on a combination of clinical, serologic, radiologic, and histopathologic findings. IgG4-RD has been reported to affect almost all organ systems. The kidney is the most frequently involved of the genitourinary organs. The most common renal manifestation of IgG4-RD is IgG4-RD tubulointerstitial nephritis, followed by membranous glomerulonephropathy and, less frequently, obstructive nephropathy involving the renal pelvis, ureter, or retroperitoneum...
September 2020: Radiographics: a Review Publication of the Radiological Society of North America, Inc
Ha Won Hwang, Jin-Seok Park, Seok Jeong, Don Haeng Lee, Suk Jin Choi
RATIONALE: IgG4-related disease (IgG4-RD) is a systemic disease that can involve various organs and is characterized by the infiltrations of IgG4-positive plasma cells and lymphocytes, fibrosis, and elevated serum IgG4 levels. IgG4-related sclerosing cholangitis (IgG4-RSC) is a subtype of IgG4-RD. No certain relationship between IgG4-RSC and cholangiocarcinoma has been established as yet, and there have been few reports of the simultaneous diagnosis of IgG4-RSC and cholangiocarcinoma...
August 21, 2020: Medicine (Baltimore)
Pradnya Brijmohan Bhattad, David L Joseph, Eric Peterson
Immunoglobulin G4-related disease (IgG4-RD) is a chronic fibrosing inflammatory systemic disorder that has been recognized relatively recently in the medical literature. Little is known about the exact disease pathogenesis and epidemiology. IgG4-RD may be asymptomatic or may have minimal symptoms or involve multiple organs with overt symptoms. The different phenotypes of IgG4-RD can lead to delayed or incorrect diagnosis. We report the case of a 66-year-old male with coal worker's pneumoconiosis who presented with progressive kidney disease and was diagnosed with tubulointerstitial nephritis due to IgG4-RD...
January 2020: Journal of Investigative Medicine High Impact Case Reports
Samar Aboulenain, Tatiana P Miquel, Juan J Maya
Immunoglobulin G4-related disease (IgG4-RD) is a new disease entity of rare and complex immune-mediated fibroinflammatory conditions that can affect any organ. The concomitance of IgG4 sclerosing sialadenitis and dacryoadenitis with rhinosinusitis is extremely rare. We report a case of IgG4 sclerosing sialadenitis and dacryoadenitis (Mikulicz's disease) diagnosed in a middle-aged African American man with a long-standing history of chronic rhinosinusitis who presented with progressively worsening bilateral salivary and lacrimal glands swelling...
August 15, 2020: Curēus
Kamonchanok Phaopraphat, Pintip Ngamjanyaporn, Pongthorn Narongroeknawin, Nuntana Kasitanon, Wanruchada Katchamart
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is an uncommon chronic systemic autoimmune disease, pathologically characterized by lymphoplasma cell and IgG4 plasma cell infiltration with storiform fibrosis. IgG4-RD is a new disease and is not yet widely recognized. The aim of this study was to describe the clinical manifestations and outcomes in Thai patients with IgG4-RD. METHODS: This multicenter retrospective cohort study included patients aged ≥ 18 years who were diagnosed with IgG4-RD, according to the 2011 comprehensive or consensus diagnostic criteria, between 2000 and 2019 in four academic centers in Thailand...
August 18, 2020: International Journal of Rheumatic Diseases
Yan Xie, Anji Xiong, Tony Marion, Yi Liu
BACKGROUND: This study was undertaken in an attempt to characterize the frequency and clinical features of lung nodules in IgG4 related disease (IgG4-RD) patients as an insight for help with the diagnosis of lung nodules. METHODS: A retrospective study was carried out in West China Hospital, Sichuan University from January 2012 to December 2018, 89 patients with definite IgG4-RD were enrolled. RESULTS: Fifty of 89 patients with definite IgG4-RD had radiologically confirmed lung nodules, 6 of whom were diagnosed with definite IgG4 related lung disease...
August 14, 2020: BMC Pulmonary Medicine
Yanying Liu, Lijuan Zhu, Zhenfan Wang, Qiaozhu Zeng, Fei Yang, Jingyuan Gao, Ziqiao Wang, Kunkun Wang, Limin Ren, Yanyan Zhang, Shanshan Zhang, Yi Wang, Danhua Shen, Changsheng Xia, Guangyan Yu, Zhanguo Li
OBJECTIVES: IgG4-related disease (IgG4-RD) is recently recognized as a fibro-inflammatory condition featured by tumefactive lesions in multiple organs, and the retroperitoneum is one of the common involved sites. We undertook this study to compare detailed demographic, clinical and laboratory characteristics of IgG4-RD patients with retroperitoneum lesion (IgG4-RD RPF+) and retroperitoneum free IgG4-RD (IgG4-RD RPF-) in a large cohort. METHODS: We carried out a retrospective review of the medical records of 407 cases of IgG4-RD diagnosed at Peking University People's Hospital between March 2009 and May 2019...
August 13, 2020: Rheumatology
Yusuke Kunimatsu, Nozomi Tani, Izumi Sato, Yuri Ogura, Kazuki Hirose, Takayuki Takeda
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a systemic immune-mediated, fibroinflammatory disorder categorized as proliferative or fibrotic depending on the responsiveness of corticosteroid treatment. IgG4-related lung disease (IgG4-RLD) accounts for 13-14% of IgG4-RD cases, but respiratory failure is quite rare. A 71-year-old man diagnosed with interstitial lung disease was referred to our department after a 10-month observational period. He presented with respiratory failure at the first visit, with significant elevations in serum IgG4 levels and histopathological findings meeting the criteria of IgG4-positive plasma cells and IgG4/IgG-positive plasma cell ratio in transbronchial lung biopsy and inguinal lymph node biopsy, resulting in a diagnosis of IgG4-RD...
October 2020: Respirology Case Reports
Hazan Karadenİz, Augusto Vaglio
Immunoglobulin G4 related disease (IgG4-RD), is an immune-mediated fibroinflammatory condition, which may involve multiple organs and mostly presents with high serum IgG4 levels and specific histopathological characteristics. As IgG4-RD is a relatively new entity the etiology, prevalence and epidemiologic knowledge is quite limited. Although involvement of almost all anatomical regions has been reported, the most commonly affected regions are pancreas, lacrimal glands, salivary glands, retroperitoneum, orbita, lymph nodes, kidney and lungs...
August 11, 2020: Turkish Journal of Medical Sciences
Katarzyna Dylewska, Katarzyna Kobusińska, Andrzej Kurylak
Introduction: IgG4-related disease (IgG4-RD) is a systemic fibrotic-inflammatory disease characterised by elevated serum concentration of IgG4 and tissue infiltration by plasma cells. IgG4-RD is a newly recognised fibro-inflammatory condition, characterised by organ mass lesions, special histopathological appearance, and - often but not always - elevated serum IgG4 concentrations. IgG4-RD is a separate, clinically distinct disease entity, but, due to its heterogeneous manifestation, it is a subject of interest of physicians of various specialties...
2020: Contemporary Oncology Współczesna Onkologia
Toshihide Tanaka, Michiyasu Fuga, Akihiko Teshigawara, Yuzuru Hasegawa, Kaichi Nishiwaki, Yuichi Murayama, Hideaki Yokoo
BACKGROUND: We report an extremely rare case of a frontal convexity tumor diagnosed as IgG4-related disease (IgG4-RD) with unique neuroradiological images. CASE DESCRIPTION: A 64-year-old man with a history of monoclonal gammopathy of undetermined significance (MGUS) and conservative follow-up presented with left facial spasm. Computed tomography showed a high-density round tumor with perifocal edema in the right frontal convexity. Magnetic resonance imaging demonstrated unique findings including low intensity on T1-, T2-, fluid-attenuated inversion recovery, and diffusion-weighted images with slight gadolinium enhancement...
August 5, 2020: World Neurosurgery
Long-Shan Ji, Xue-Hua Sun, Xin Zhang, Zhen-Hua Zhou, Zhuo Yu, Xiao-Jun Zhu, Ling-Ying Huang, Miao Fang, Ya-Ting Gao, Man Li, Yue-Qiu Gao
Helping B cells and antibody responses is a major function of CD4+ T helper cells. Follicular helper T (Tfh) cells are identified as a subset of CD4+ T helper cells, which is specialized in helping B cells in the germinal center reaction. Tfh cells express high levels of CXCR5, PD-1, IL-21, and other characteristic markers. Accumulating evidence has demonstrated that the dysregulation of Tfh cells is involved in infectious, inflammatory, and autoimmune diseases, including lymphocytic choriomeningitis virus (LCMV) infection, inflammatory bowel disease (IBD), systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), IgG4-related disease (IgG4-RD), Sjögren syndrome (SS), and type 1 diabetes (T1D)...
2020: Journal of Immunology Research
Eytan Keidar, Jacob Shermetaro, Gary Kwartowitz
Chronic sclerosing sialadenitis (CSS) or Küttner tumor is an under-recognized, benign fibroinflammatory disease most commonly seen in the submandibular gland of older adult males. Sialolithiasis or bacterial infection was first suspected as an etiology, but CSS is now considered an immunoglobulin G4-related disease (IgG4-RD). IgG4-RD can affect almost every organ in the body, characterized by organ fibrosis with IgG4-positive plasmacytes. Numerous autoimmune-related diseases have been unified under IgG4-RD, including Mikulicz disease (MD), autoimmune pancreatitis, Reidel's thyroiditis, and others...
June 26, 2020: Curēus
Satomi Kasashima, Atsuhiro Kawashima, Fuminori Kasashima, Yasushi Matsumoto, Satoru Ozaki
A 78-year-old male was admitted to our hospital with lumbar pain and was found to have an abdominal aortic aneurysm (AAA) and femoral artery aneurysm (FAA). Initially, the patient underwent endovascular aneurysm repair (EVAR) for the AAA and aneurysmectomy for the FAA. The FAA was diagnosed by histology as immunoglobulin G4-related disease (IgG4-RD). The preoperative serum IgG4 level was within the normal range, although a slight serum interleukin-6 (IL-6) elevation was observed. Four years later, the AAA-sac diameter had expanded and the serum levels of both IgG4 and IL-6 levels had increased...
August 4, 2020: Pathology International
Tomoyuki Ueki, Toru Miyake, Mitsuhiro Narita, Masatsugu Kojima, Sachiko Kaida, Hiroya Iida, Tomoharu Shimizu, Masaji Tani
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a novel disease concept of unknown cause that is characterized by abundant infiltration of IgG4-positive cells, mass-forming lesions, and elevated serum IgG4 levels. The infiltration of IgG4-positive plasma cells and lymphocytes causes swelling, inflammation, fibrosis, and obliterative phlebitis in multiple organs. On the other hand, IgG4-RD occurring in the ureters has rarely been reported. To our knowledge, this is the first report of laparoscopic partial ureteral resection for IgG4-related focal retroperitoneal fibrosis in a ureter with suspected colon cancer recurrence...
August 3, 2020: Surgical Case Reports
Darren Kastin, Matthew Siegel, Richard Anderson, Andrew Aronsohn
Autoimmune Hepatitis (AIH) and Idiopathic Hypereosinophilia Syndrome (HES) are viewed as distinct diseases. Our patient presented with hepatic and serum IgG4+ plasma cell infiltration, in addition to a marked bone marrow and peripheral eosinophilia with colonic involvement, raising the diagnosis of IgG4-related disease (IgG4-RD) and HES. This is the first reported case of IgG4 - AIH presenting with HES, both of which resolved with immune suppressive therapy.
August 3, 2020: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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