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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/29229107/clinical-characterization-of-52-patients-with-immunoglobulin-g4-related-disease-in-a-single-tertiary-center-in-japan-special-reference-to-lung-disease-in-thoracic-high-resolution-computed-tomography
#1
Takeshi Saraya, Kosuke Ohkuma, Masachika Fujiwara, Chika Miyaoka, Shoko Wada, Takayasu Watanabe, Sunao Mikura, Manami Inoue, Miku Oda, Mitsuru Sada, Yukari Ogawa, Kojiro Honda, Masaki Tamura, Takuma Yokoyama, Daisuke Kurai, Haruyuki Ishii, Hajime Takizawa
BACKGROUND: Immunoglobulin G4-related disease (IgG4-RD) is a rare multi-organ disorder. Physicians rarely encounter patients with IgG4-RD and its range of symptoms. METHODS: To elucidate the clinical characterization of IgG4-RD, along with the clinical significance of lung involvement, we retrospectively reviewed the medical records of patients who satisfied the comprehensive diagnostic criteria for IgG4-RD. RESULTS: We identified 52 patients with IgG4-RD...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29229083/thoracic-involvement-in-igg4-related-disease-in-a-uk-based-patient-cohort
#2
John P Corcoran, Emma L Culver, Rebekah M Anstey, Ambika Talwar, Charis D Manganis, Tamsin N Cargill, Robert J Hallifax, Ioannis Psallidas, Najib M Rahman, Eleanor Barnes
IgG4-related disease (IgG4-RD) is a multi-system fibro-inflammatory disorder with classical histopathological findings, often in the context of elevated serum IgG4 levels. The thoracic manifestations of IgG4-RD are numerous and can mimic several common and better known conditions. The objective of this study was to outline the frequency and nature of thoracic involvement in a prospective cohort of IgG4-RD patients who met defined diagnostic criteria. Over 40% of IgG4-RD patients had clinicoradiological and/or histological evidence of thoracic involvement, predominantly mediastinal lymphadenopathy, the majority associated with multi-system disease outside the chest...
November 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29222587/gastrointestinal-manifestation-of-immunoglobulin-g4-related-disease-clarification-through-a-multicenter-survey
#3
Kenji Notohara, Terumi Kamisawa, Kazushige Uchida, Yoh Zen, Mitsuhiro Kawano, Satomi Kasashima, Yasuharu Sato, Masahiro Shiokawa, Takeshi Uehara, Hajime Yoshifuji, Hiroko Hayashi, Koichi Inoue, Keisuke Iwasaki, Hiroo Kawano, Hiroyuki Matsubayashi, Yukitoshi Moritani, Katsuhiko Murakawa, Yoshio Oka, Masatoshi Tateno, Kazuichi Okazaki, Tsutomu Chiba
BACKGROUND: Several reports on immunoglobulin (Ig)G4-related disease (IgG4-RD) with gastrointestinal involvement (IgG4-related gastrointestinal disease; IgG4-GID) have been published, although this entity has not been fully established clinicopathologically. Thus, we carried out a multicenter survey. METHODS: Patients with possible IgG4-GID who underwent resection were collected. Histologic slides were reevaluated, and eight cases with diffuse lymphoplasmacytic infiltration but without numerous neutrophils, granulations or epithelioid granulomas were further analyzed...
December 8, 2017: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29204759/ocular-manifestations-of-igg4-related-disease-in-children-more-common-than-anticipated-review-of-the-literature-and-case-report
#4
REVIEW
Roubini G Smerla, Dimitra Rontogianni, George E Fragoulis
IgG4-related disease (IgG4-RD) is an entity with various clinical manifestations. Histopathologically, it is characterized by lymphoplasmacytic infiltrates enriched in IgG4 (+) plasmacytes and usually fibrosis of the affected tissue. Most of the patients have also increased IgG4 serum levels and they respond to glucocorticosteroids. In children, due to its rare occurrence, IgG4-RD is ill defined. From the published studies, so far, it appears that ocular manifestations are very common in the paediatric population with IgG4-RD...
December 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29196804/igg4-related-disease-with-emphasis-on-the-biopsy-diagnosis-of-autoimmune-pancreatitis-and-sclerosing-cholangitis
#5
REVIEW
Sönke Detlefsen, Günter Klöppel
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts...
December 1, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29191210/new-clues-to-the-nature-of-immunoglobulin-g4-related-disease-a-retrospective-japanese-multicenter-study-of-baseline-clinical-features-of-334-cases
#6
Kazunori Yamada, Motohisa Yamamoto, Takako Saeki, Ichiro Mizushima, Shoko Matsui, Yuhei Fujisawa, Satoshi Hara, Hiroki Takahashi, Hideki Nomura, Shigeyuki Kawa, Mitsuhiro Kawano
BACKGROUND: The aim was to further characterize immunoglobulin G4-related disease (IgG4-RD) by a large-scale multicenter study of its clinical and laboratory features conducted by multidisciplinary physicians of IgG4-RD in Japan. METHODS: Various specialists retrospectively evaluated IgG4-RD patients diagnosed between 1996 and 2015 in five hospitals by analyzing their baseline clinical features, laboratory, imaging, and pathological test findings, and treatment...
December 1, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29155962/igg4-related-disease-presenting-with-raised-serum-igg2-real-timeline-of-igg4-rd
#7
Lisa Dunkley, Hardeep S Mudhar
No abstract text is available yet for this article.
October 9, 2017: Rheumatology
https://www.readbyqxmd.com/read/29151498/early-detection-and-intervention-of-coronary-artery-involvement-in-immunoglobulin-g4-related-disease
#8
Yoji Komiya, Makoto Soejima, Daisuke Tezuka, Hitoshi Kohsaka
A 59-year-old man with swollen submandibular glands developed an aortic aneurysm requiring aortic prosthesis implantation. Echocardiography performed to evaluate the cardiac function before the surgery incidentally revealed masses around the coronary arteries. The serum IgG4 levels were increased. A post-operational pathological examination of the abdominal aneurysms revealed infiltration of plasma cells, with the ratio of IgG4/IgG-positive cells being >80%. The patient was diagnosed with IgG4-related disease (RD) with coronary artery involvement...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29151297/the-clinical-features-and-outcomes-of-turkish-patients-with-igg4-related-disease-a-single-center-experience
#9
Ömer Karadağ, Abdulsamet Erden, Emine Arzu Ayhan, Ertuğrul Çağrı Bölek, Umut Kalyoncu, Berkan Armağan, Alper Sarı, Levent Kılıç, Ali Akdoğan, Tuncay Hazırolan, Bülent Akdoğan, Şaziye Şule Apraş Bilgen, Dilek Baydar, Sedat Kiraz, Ali İhsan Ertenli
Background/aim: Since the majority of the IgG4-related disease (IgG4-RD) patients in the literature are from the Far East and the United States, there is a lack of large series from other parts of the world. We aimed to identify the clinical characteristics and outcome of Turkish IgG4-RD patients from a tertiary center. Materials and methods: Fifty-two patients classified as having definite IgG4-RD according to comprehensive diagnostic criteria were included in the study. Patients not fulfilling the definite criteria due to lack of pathologic specimen and/or serum IgG4 levels were excluded (n = 47)...
November 13, 2017: Turkish Journal of Medical Sciences
https://www.readbyqxmd.com/read/29105322/clinical-characteristics-and-outcomes-of-61-patients-with-chronic-periaortitis-including-igg4-related-and-non-igg4-related-cases
#10
In Young Kim, Yeong Hee Eun, Hyemin Jeong, Taek Kyu Park, Hyungjin Kim, Jaejoon Lee, Shin Yi Jang, Jung-Sun Kim, Eun-Mi Koh, Duk-Kyung Kim, Hoon-Suk Cha
AIM: Chronic periaortitis (CP) is a disease characterized by a fibro-inflammatory periaortic cuff and adventitia-predominant fibrosis. CP encompasses idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysm (AAA), and recent studies have documented overlap between CP and immunoglobulin G4-related disease (IgG4-RD). This study aimed to investigate clinical characteristics and treatment outcomes of patients with CP. METHOD: CP patients were identified by retrospective review of 1245 patients with International Classification of Diseases 10th edition code of aortitis or aortic disease...
November 3, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29099539/igg4-related-disease-in-thymus-a-very-rare-case-of-chronic-fibrosis-mimicking-sarcoidosis
#11
Sara Simonetti, Noelia Pérez Muñoz, Josefa López Vivancos, Lluís Sanchez Sitjes, Javier Cesar Herranz Pérez, Nelson Leal Bohorquez, José Antonio Maestre Alcacer, Inessa Koptseva de García, Miguel Ángel Carrasco García
IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally...
October 28, 2017: Tumori
https://www.readbyqxmd.com/read/29072949/igg4-related-disease-a-reminder-for-practicing-pathologists
#12
REVIEW
Steven C Weindorf, John Karl Frederiksen
IgG4-related disease (IgG4-RD) is a systemic autoimmune fibroinflammatory disease that produces sclerotic, tumefactive masses containing dense lymphoplasmacytic infiltrates rich in immunoglobulin (Ig) G4+ plasma cells. Initially characterized as a form of autoimmune pancreatitis, the distinctive histopathology of IgG4-RD has now been described in almost every organ system. However, because the clinical manifestations of IgG4-RD are diverse and nonspecific, the disease may go unsuspected until a biopsy or resection specimen is obtained to diagnose a presumed malignancy...
November 2017: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/29067928/multi-organ-igg4-related-disease-demystifying-the-diagnostic-enigma
#13
S Bhardwaj, S Goyal, A K Yadav, A Goyal
IgG4-related disease (IgG4-RD) is a multisystemic mass forming immune-mediated disease entity, commonly creating confusion and diagnostic challenges. We present a case of a 25-year-old female who presented with bilateral orbital masses, lymphadenopathy, paraspinal and renal masses, which clinicoradiologically simulated lymphoma. The lymph node biopsy revealed interfollicular sheets of plasma cells creating confusion with Castleman's disease and marginal zone lymphoma. The orbital biopsy revealed ductular destruction, periductular plasma cells, and fibrosis, mimicking Sjogren's syndrome and Castleman's disease...
October 23, 2017: Journal of Postgraduate Medicine
https://www.readbyqxmd.com/read/29063068/immunoglobulin-g4-related-kidney-disease-pathogenesis-diagnosis-and-treatment
#14
REVIEW
Ke Zheng, Fei Teng, Xue-Mei Li
Immunoglobulin G4-related disease (IgG4-RD) is a recently recognized clinical entity that often involves multiple organs; it is characterized by high levels of serum immunoglobulin G4 (IgG4), dense infiltration of IgG4+ cells, and storiform fibrosis. Cellular immunity, particularly T cell-mediated immunity, has been implicated in the pathogenesis of IgG4-RD. The most frequent renal manifestations of IgG4-RD are IgG4-related tubulointerstitial nephritis, membranous glomerulonephropathy (MGN), and obstructive nephropathy secondary to urinary tract obstruction due to IgG4-related retroperitoneal fibrosis, prostatitis, or ureter inflammation...
September 2017: Chronic diseases and translational medicine
https://www.readbyqxmd.com/read/29061247/neurologic-features-of-immunoglobulin-g4-related-disease
#15
REVIEW
Mahmoud AbdelRazek, John H Stone
Immunoglobulin G4-related disease (IgG4-RD) can involve nearly any organ system, including the central and peripheral nervous systems. IgG4 antibodies are not known to play a primary etiologic role in disease. IgG4-RD must be distinguished from a growing number of immune-mediated conditions in which IgG4 autoantibodies contribute directly to pathophysiology. The most common neurologic features of IgG4-RD result from disease in the orbits, pachymeninges, and substance of the pituitary gland and stalk, as well as a perineuropathy that can involve peripheral or cranial nerves...
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29046807/pulmonary-schistosomiasis-mimicking-igg4-related-lung-disease
#16
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
#17
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)...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28987046/-igg4-related-disease-a-case-report
#18
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
#19
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
#20
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
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