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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/29046807/pulmonary-schistosomiasis-mimicking-igg4-related-lung-disease
#1
Timothy Baird, Caroline L Cooper, Richard Wong, Naomi Runnegar, Gregory Keir
IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory disease characterized by tumefactive lesions in various organ systems, including the lungs. Clinical and radiological manifestations of IgG4-RD are relatively non-specific, and we report a case highlighting the importance of histopathological confirmation in cases of suspected IgG4-related lung disease. A 44-year-old male with significantly elevated serum IgG4 levels, patchy consolidation on thoracic CT imaging, and cough was referred with suspected IgG4-related lung disease...
January 2018: Respirology Case Reports
https://www.readbyqxmd.com/read/29039547/frequency-and-distribution-of-cd4-cxcr5-follicular-b-helper-t%C3%A2-cells-within-involved-tissues-in-igg4%C3%A2-related-ophthalmic-disease
#2
Huimin Yang, Ruili Wei, Qiang Liu, Yongheng Shi, Jin Li
Immonoglobulin G4‑related ophthalmic disease (IgG4‑ROD) is a IgG4‑RD and exhibits two main characteristics: Fibrosis that is not necessarily marked histopathologically; and frequent formation of germinal centers (GCs). Follicular B helper T (Tfh) cells are now recognized as the true helper cells for B cells in antibody responses. In the present study, the profile and distribution of Tfh cells in involved tissues from patients with IgG4‑ROD was compared to those of type 1 autoimmune pancreatitis (AIP) and patients with IgG4‑related lymphadenopathy (IgG4‑RL)...
October 12, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28987046/-igg4-related-disease-a-case-report
#3
Justyna Milczarek-Banach, Kinga Brodzińska, Anna Jankowska, Urszula Ambroziak, Benedykt Szczepankiewicz, Jolanta Nałęcz-Janik, Piotr Miśkiewicz
Immunoglobulin G4-related disease (IgG4-RD) is a comparatively new condition that may involve more than one organ. The lack of characteristic, pathognomonic clinical symptoms may delay the diagnosis of this disease. The diagnosis is based upon clinical manifestation, elevated serum levels of IgG4 and histopathologic examination with immunohistochemical staining to reveal infiltration of IgG4-positive plasma cells. The first line treatment is oral glucocorticoids. A CASE REPORT: 38-year-old woman with Hashimoto disease, chronic sinusitis and chronic hepatitis of unknown etiology was admitted to the Department of Endocrinology because of moderate eyelids swelling accompanied by redness for 3 years...
September 29, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28978347/clinical-features-of-igg4-related-periaortitis-periarteritis-based-on-the-analysis-of-179-patients-with-igg4-related-disease-a-case-control-study
#4
Makiko Ozawa, Yasunari Fujinaga, Junpei Asano, Akira Nakamura, Takayuki Watanabe, Tetsuya Ito, Takashi Muraki, Hideaki Hamano, Shigeyuki Kawa
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized systemic condition characterized by high serum immunoglobulin G4 (IgG4) concentration and IgG4-bearing plasma cell infiltration in affected organs. Although it has become evident that IgG4-RD also involves the systemic aortic/arterial system, the precise details of this condition remain unclear. The present study sought to clarify the clinical features of IgG4-related periaortitis/periarteritis. METHODS: Among 223 patients with IgG4-RD, 179 (131 male, median onset age 67 years) were recruited for this study...
October 4, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28977663/quantitative-measurement-of-18f-fdg-pet-ct-uptake-reflects-the-expansion-of-circulating-plasmablasts-in-igg4-related-disease
#5
Alvise Berti, Emanuel Della-Torre, Francesca Gallivanone, Carla Canevari, Raffaella Milani, Marco Lanzillotta, Corrado Campochiaro, Giuseppe Alvise Ramirez, Emanuele Bozzalla Cassione, Enrica Bozzolo, Federica Pedica, Isabella Castiglioni, Paolo Giorgio Arcidiacono, Gianpaolo Balzano, Massimo Falconi, Luigi Gianolli, Lorenzo Dagna
Objective: [ 18 F]Fluorodeoxyglucose ( 18 F-FDG) PET/CT is increasingly used to assess organ involvement and response to treatment in IgG4-related disease (IgG4-RD), but clear correlations between 18 F-FDG uptake and disease activity have not been established yet. We aimed to correlate the intensity and distribution of 18 F-FDG uptake with validated clinical, serological and immunological parameters of IgG4-RD activity. Methods: Twenty patients with active IgG4-RD underwent a baseline 18 F-FDG PET/CT...
July 7, 2017: Rheumatology
https://www.readbyqxmd.com/read/28959455/distinct-features-distinguishing-igg4-related-disease-from-multicentric-castleman-s-disease
#6
Takanori Sasaki, Mitsuhiro Akiyama, Yuko Kaneko, Takehiko Mori, Hidekata Yasuoka, Katsuya Suzuki, Kunihiro Yamaoka, Shinichiro Okamoto, Tsutomu Takeuchi
OBJECTIVES: Differentiating IgG4-related disease (IgG4-RD) from multicentric Castleman's disease (MCD) is challenging because both diseases present high serum IgG4. The objective of this study is to clarify the differences in characteristics and identify a clinically useful approach to differentiate these two diseases. METHODS: Forty-five consecutive patients with untreated active IgG4-RD and 33 patients with MCD were included in this study, who visited our institution from January 2000 to August 2016...
2017: RMD Open
https://www.readbyqxmd.com/read/28958166/igg4-related-disease-presenting-as-intradural-extramedullary-lesion-a-case-report-and-review-of-the-literature
#7
Kelly J Bridges, Caitlin H DeDeaux, Khoi D Than
OBJECT: IgG4-related disease (IgG4-RD) is a fibro-inflammatory disorder affecting various anatomical sites, and only recently was identified to affect the dura of the spine. The authors present the second reported case of an intradural extramedullary lesion consistent with IgG4-related spinal disease. METHODS: A literature review was performed that identified 15 other cases of spinal disease, and common features of all known reported spinal IgG4-RD are discussed...
September 28, 2017: British Journal of Neurosurgery
https://www.readbyqxmd.com/read/28934889/clinical-outcomes-after-endovascular-repair-and-open-surgery-to-treat-immunoglobulin-g4-related-and-nonrelated-inflammatory-abdominal-aortic-aneurysms
#8
Satomi Kasashima, Fuminori Kasashima, Atsuhiro Kawashima, Masamitsu Endo, Yasushi Matsumoto, Kengo Kawakami
PURPOSE: To compare the follow-up results of endovascular aneurysm repair (EVAR) vs open surgery (OS) for inflammatory abdominal aortic aneurysms (IAAAs) with regard to immunoglobulin G4-related diseases (IgG4-RD), which are fibrous inflammatory conditions characterized by elevated serum IgG4 concentrations and numerous infiltrations of IgG4(+) plasmacytes. METHODS: Between January 2005 and December 2015, 91 patients were treated with EVAR (begun in 2008) and 166 patients underwent OS for AAA...
September 1, 2017: Journal of Endovascular Therapy
https://www.readbyqxmd.com/read/28922375/elevated-igg4-serum-levels-in-patients-with-cystic-fibrosis
#9
Axelle Clerc, Quitterie Reynaud, Stéphane Durupt, Colette Chapuis-Cellier, Raphaële Nové-Josserand, Isabelle Durieu, Jean Christophe Lega
OBJECTIVE: Serum immunoglobulin (Ig) G4 elevation has been associated with several pathological conditions other than IgG4-related disease (IgG4-RD). In cystic fibrosis (CF), an elevation of specific IgG4 has been associated with colonization and infection by Pseudomonas aeruginosa. IgG4 elevation may be a marker of chronic infection or inflammatory stimulation. The aim of this study was to explore the prevalence of elevated IgG4 levels in CF and its correlation with the major clinical and microbiological features found in CF patients...
2017: PloS One
https://www.readbyqxmd.com/read/28918831/a-comparative-analysis-of-serum-igg4-levels-in-patients-with-igg4-related-disease-and-other-disorders
#10
Li Wang, Xinmin Chu, Yan Ma, Min Zhang, Xue Wang, Li Jin, Zhen Tan, Xiangpei Li, Xiaomei Li
OBJECTIVE: Elevated serum IgG4 levels are an important hallmark for diagnosing IgG4-related disease (IgG4-RD) but can also be found and reported in other diseases. The present study intended to compare the serum IgG4 levels in both IgG4-RD and non-IgG4-RD and determine the serum IgG4 levels in patients with IgG4-RD before and after glucocorticoid therapy. METHODS: The study included 323 patients from Anhui Medical University Affiliated Provincial Hospital (China) and was conducted from July 2014-January 2016...
September 2017: American Journal of the Medical Sciences
https://www.readbyqxmd.com/read/28916523/cutting-edge-a-critical-role-of-lesional-t-follicular-helper-cells-in-the-pathogenesis-of-igg4-related-disease
#11
Ryuta Kamekura, Kenichi Takano, Motohisa Yamamoto, Koji Kawata, Katsunori Shigehara, Sumito Jitsukawa, Tomonori Nagaya, Fumie Ito, Akinori Sato, Noriko Ogasawara, Chieko Tsubomatsu, Hiroki Takahashi, Hiroshi Nakase, Tetsuo Himi, Shingo Ichimiya
IgG4-related disease (IgG4-RD) is a newly recognized systemic chronic fibroinflammatory disease. However, the pathogenesis of IgG4-RD remains unknown. To determine the pathophysiologic features of IgG4-RD, we examined T follicular helper (Tfh) cells in lesions and blood from patients with IgG4-RD. Patients with IgG4-related dacryoadenitis and sialadenitis (IgG4-DS) showed increased infiltration of Tfh cells highly expressing programmed death 1 and ICOS in submandibular glands. Tfh cells from IgG4-DS submandibular glands had higher expression of B cell lymphoma 6 and a greater capacity to help B cells produce IgG4 than did tonsillar Tfh cells...
October 15, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28915275/long-term-efficacy-and-safety-of-rituximab-in-igg4-related-disease-data-from-a-french-nationwide-study-of-thirty-three-patients
#12
MULTICENTER STUDY
Mikael Ebbo, Aurélie Grados, Maxime Samson, Matthieu Groh, Anderson Loundou, Aude Rigolet, Benjamin Terrier, Constance Guillaud, Clarisse Carra-Dallière, Frédéric Renou, Agnieszka Pozdzik, Pierre Labauge, Sylvain Palat, Jean-Marie Berthelot, Jean-Loup Pennaforte, Alain Wynckel, Céline Lebas, Noémie Le Gouellec, Thomas Quémeneur, Karine Dahan, Franck Carbonnel, Gaëlle Leroux, Antoinette Perlat, Alexis Mathian, Patrice Cacoub, Eric Hachulla, Nathalie Costedoat-Chalumeau, Jean-Robert Harlé, Nicolas Schleinitz
OBJECTIVES: To assess efficacy and safety of rituximab (RTX) as induction therapy, maintenance of remission and treatment of relapses in a cohort of IgG4-related disease (IgG4-RD) patients. METHODS: Nationwide retrospective multicenter study of IgG4-RD patients treated with at least one course of RTX. Clinical, biological and radiological response, relapse rate and drug tolerance were analyzed. Kaplan-Meier curves were plotted and risk factors for relapse studied with a Cox regression model...
2017: PloS One
https://www.readbyqxmd.com/read/28912998/igg4-related-kidney-disease-report-of-a-case-presenting-as-a-renal-mass
#13
Daniele Bianchi, Luca Topazio, Gabriele Gaziev, Valerio Iacovelli, Pierluigi Bove, Alessandro Mauriello, Enrico Finazzi Agrò
IgG4-related disease (IgG4-RD) is a nosological entity defined as a chronic immune-mediated fibro-inflammatory condition characterized by a tendency to form tumefactive, tissue-destructive lesions or by organ failure. Urologic involvement in IgG4-RD has been described in some short series of patients and in isolated case reports, most often involving the kidneys in so-called IgG4-related kidney disease (IgG4-RKD). The disease can occasionally mimic malignancies and is at risk of being misdiagnosed due to its rarity...
2017: Case Reports in Surgery
https://www.readbyqxmd.com/read/28879714/serum-igg4-concentration-in-igg4-related-disease
#14
REVIEW
Shigeyuki Kawa, Markus Skold, David B Ramsden, Antony Parker, Stephen J Harding
BACKGROUND: IgG4-related disease (IgG4-RD) is an immune-mediated and chronic fibroinflammatory condition that affects almost any organ and often involves multiple organs in the same patient. In this review article, we address the clinical utility of measuring serum immunoglobulin G subclass 4 concentration ([IgG4]) in IgG4-RD diagnosis and in disease monitoring. METHODS: We discuss the latest literature on the relevance of [IgG4] to the investigation and management of IgG4RDs...
September 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#15
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28859705/immunoglobulin-g4-sinusitis-in-association-with-aspirin-exacerbated-respiratory-disease
#16
Kirti Johal, Kevin Welch, Anju Peters
BACKGROUND: Immunoglobulin G4 (IgG4) related disease is a systemic inflammatory disease characterized by tumor-like tissue infiltration with IgG4 positive (IgG4+) plasma cells. Aspirin-exacerbated respiratory disease (AERD) is defined as asthma, chronic rhinosinusitis with nasal polyposis, and hypersensitivity to cyclooxygenase-1 inhibitors. OBJECTIVE: We described a case of a non-smoking 61-year-old male with prior NSAID sensitivity who presented with a 1-year history of left eye proptosis associated with chronic nasal symptoms, ultimately identified as concurrent AERD and IgG4 sinusitis...
September 1, 2017: American Journal of Rhinology & Allergy
https://www.readbyqxmd.com/read/28858963/igg4-related-disease-in-the-eye-and-ocular-adnexa
#17
REVIEW
Larissa Derzko-Dzulynsky
PURPOSE OF REVIEW: IgG4-related disease is a multi-organ fibro-inflammatory disease with characteristic histopathology showing lymphoplasmacytic infiltration, increased IgG4+ plasma cells and elevated IgG4/IgG ratios (>40%). The lacrimal gland is the most common ocular site of involvement. Scleritis and intraocular involvement in IgG4-related ophthalmic disease (IgG4-ROD) have recently been reported. The purpose of this review is to describe orbital and intraocular IgG4-ROD with a focus on publications since 2016...
November 2017: Current Opinion in Ophthalmology
https://www.readbyqxmd.com/read/28856463/the-variety-of-clinical-presentations-in-igg4-related-disease-in-rheumatology
#18
REVIEW
Agata Sebastian, Maciej Sebastian, Maria Misterska-Skóra, Piotr Donizy, Agnieszka Hałoń, Arkadiusz Chlebicki, Artur Lipiński, Piotr Wiland
IgG4-related disease (IgG4-RD) belongs to the group of rare diseases in which the identification of the characteristic histology and immunohistochemistry provides with the gold standard in the diagnosis. The variable organ dysfunction reflects the clinical presentation. The examples of different IgG4-RD presentations in the Rheumatology Unit were discussed in this article. The spectrum of IgG4-RD is wide-ranging and manifested in one or more organs synchronously or metachronously. In the presented article, we described five different cases of IgG4-RD...
August 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28830675/expansion-of-blood-igg4-b-th2-and-regulatory-t-cells-in-patients-with-igg4-related-disease
#19
Jorn J Heeringa, A Faiz Karim, Jan A M van Laar, Robert M Verdijk, Dion Paridaens, P Martin van Hagen, Menno C van Zelm
BACKGROUND: IgG4-related disease (IgG4-RD) is a systemic fibroinflammatory condition affecting various organs and has a diverse clinical presentation. Fibrosis and accumulation of IgG4(+) plasma cells in tissue are hallmarks of the disease, and IgG4-RD is associated with increased IgG4 serum levels. However, disease pathogenesis is still unclear, and these cellular and molecular parameters are neither sensitive nor specific for the diagnosis of IgG4-RD. OBJECTIVE: Here we sought to develop a flow cytometric gating strategy to reliably identify blood IgG4(+) B cells to study their cellular and molecular characteristics and investigate their contribution in disease pathogenesis...
August 19, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28829660/the-impact-of-igg-4-rod-on-the-diagnosis-of-orbital-tumors-a-retrospective-analysis
#20
Aleza Andron, Avner Hostovsky, Akshay Gopinathan Nair, Oded Sagiv, Ginette Schiby, Guy Ben- Simon
This study was to determine the prevalence of immunoglobulin G4 (IgG4)-related orbital disease (IgG4-ROD) among patients who have previously undergone biopsy and were diagnosed to have idiopathic orbital inflammatory disease (IOID) or orbital lymphoproliferative disease (OLD), namely, lymphoma and benign reactive lymphoid hyperplasia (BRLH). This is a retrospective cross-sectional study. The charts and slides of all patients who underwent biopsies and were histopathologically diagnosed to have either IOID or OLD were reviewed...
August 22, 2017: Orbit
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