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"IgG4-related disease"

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https://www.readbyqxmd.com/read/28928271/the-value-of-18-f-fdg-pet-ct-in-identifying-the-cause-of-fever-of-unknown-origin-fuo-and-inflammation-of-unknown-origin-iuo-data-from-a-prospective-study
#1
Verena Schönau, Kristin Vogel, Matthias Englbrecht, Jochen Wacker, Daniela Schmidt, Bernhard Manger, Torsten Kuwert, Georg Schett
BACKGROUND: Fever of unknown origin (FUO) and inflammation of unknown origin (IUO) are diagnostically challenging conditions. Diagnosis of underlying disease may be improved by (18)F-fluorodesoxyglucose positron emission tomography ((18)F-FDG-PET). METHODS: Prospective study to test diagnostic utility of (18)F-FDG-PET/CT in a large cohort of patients with FUO or IUO and to define parameters that increase the likelihood of diagnostic (18)F-FDG-PET/CT. Patients with FUO or IUO received (18)F-FDG-PET/CT scanning in addition to standard diagnostic work-up...
September 19, 2017: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/28922443/primary-hepatic-lymphoma-complicated-by-a-hepatic-inflammatory-pseudotumor-and-tumor-forming-pancreatitis
#2
Rena Kaneko, Hiroyuki Mitomi, Natsuko Nakazaki, Yuichiro Yano, Masazumi Ogawa, Yuzuru Sato
BACKGROUND: Hepatic inflammatory pseudotumor (IPT) is considered to be benign in biological behavior, and its malignant transformation is extremely rare. There has only been one published case of primary hepatic lymphoma complicated by hepatic IPT. CASE PRESENTATION: A 73-year-old man presented with obstructive jaundice and a pancreatic head mass. Histology of the mass revealed chronic pancreatitis with lymphoid follicle formation, leading to a diagnosis of a suspicion of follicular pancreatitis...
September 2017: Journal of Gastrointestinal and Liver Diseases: JGLD
https://www.readbyqxmd.com/read/28922375/elevated-igg4-serum-levels-in-patients-with-cystic-fibrosis
#3
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/28922106/igg4-related-disease-a-patient-with-multiple-organ-involvement
#4
Miroslav Průcha, Lenka Sedláčková
IgG4-related diseases represent a heterogeneous group of conditions characterised by elevated serum IgG4 levels and fibrotic or sclerosing changes in the affected organs or systems accompanied by IgG4-positive plasma cells. A disease associated with IgG4 may affect virtually any organ - salivary glands, periorbital tissue, kidneys, lungs, meninges, aorta, prostate, pericardium or skin. Histopathological findings are uniform, characterised by a major lymphoplasmocytic infiltrate and the presence of IgG4-producing plasma cells, irrespective of the affected site...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28918831/a-comparative-analysis-of-serum-igg4-levels-in-patients-with-igg4-related-disease-and-other-disorders
#5
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/28918828/crescentic-glomerulonephritis-with-immunoglobulin-g4-related-disease
#6
Inbar Raber, Asiri Ediriwickrema, John Higgins, Neeraja Kambham, Alan C Pao
Immunoglobulin G4 (IgG4)-related disease is an uncommon autoimmune disease that affects multiple organ systems. Renal involvement typically presents as tubulointerstitial nephritis and less commonly as membranous glomerulonephritis. In this case report, we discuss a 68-year-old patient who presented with rapidly progressive glomerulonephritis. His renal biopsy revealed a membranoproliferative pattern of injury with fibrocellular crescents and extensive infiltration of the tubulointerstitium with IgG4-positive plasma cells...
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
#7
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...
September 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
#8
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
#9
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/28910817/the-lung-in-dysregulated-states-of-humoral-immunity
#10
Yurdagül Uzunhan, Florence Jeny, Marianne Kambouchner, Morgane Didier, Diane Bouvry, Hilario Nunes, Jean-François Bernaudin, Dominique Valeyre
In common variable immunodeficiency, lung manifestations are related to different mechanisms: recurrent pneumonias due to encapsulated bacteria responsible for diffuse bronchiectasis, diffuse infiltrative pneumonia with various patterns, and lymphomas, mostly B cell extranodal non-Hodgkin type. The diagnosis relies on significant serum Ig deficiency and the exclusion of any primary or secondary cause. Histopathology may be needed. Immunoglobulin (IgG) replacement is crucial to prevent infections and bronchiectasis...
September 15, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#11
Kyle Geary, Cemal Yazici, Anita Seibold, Grace Guzman
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy...
September 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28898148/igg4-related-disease-as-the-differential-diagnosis-of-hypophysitis
#12
Oscar Mp Jolobe
No abstract text is available yet for this article.
September 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28881885/igg4-related-disease-involving-the-esophagus-a-clinicopathological-study
#13
I Obiorah, A Hussain, C Palese, N Azumi, S Benjamin, M Ozdemirli
Immunoglobulin G4 (IgG4)-related disease is a recently coined systemic disease characterized by specific histopathologic findings of an intense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis in the presence of predominant IgG4-positive plasma cells. Although IgG4-related disease has been described in many organs, involvement of the esophagus is very rare. In this study, we describe the clinicopathologic characteristics of eight patients with IgG4-related esophagitis. We evaluated chronic esophagitis specimens with lymphoplasmacytic infiltrate obtained over the past 6 years (from January 2011 to February 2017) using a chart review, pathologic examination, and IgG4 immunohistochemical staining...
December 1, 2017: Diseases of the Esophagus: Official Journal of the International Society for Diseases of the Esophagus
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/28871762/igg4-related-disease
#15
Jens Vikse, Sven Håland, Katrine Brække Norheim
No abstract text is available yet for this article.
September 5, 2017: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#16
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/28868020/erdheim-chester-disease-the-importance-of-information-integration
#17
Anna Nikonova, Khashayar Esfahani, Guillaume Chausse, Stephan Probst, Tina Petrogiannis-Haliotis, Hans Knecht, Genevieve Gyger
BACKGROUND: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis disorder that utilizes the RAS-RAF-MEK-ERK pathway. It has a highly variable clinical presentation, where virtually any organ can be involved, thus having the potential of posing a great diagnostic challenge. Over half of the reported cases have the BRAF V600E mutation and have shown a remarkable response to vemurafenib. CASE PRESENTATION: We describe herein a patient with a history of stroke-like symptoms and retroperitoneal fibrosis that on initial pathology raised the possibility of IgG4-related disease...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28862040/lymphoplasmacyte-rich-meningioma-with-atypical-angiomatous-feature-and-an-increased-deposition-of-igg4-positive-plasma-cells-an-unusual-case-report
#18
Hyun-Jin Son, In Kyu Yu, Seong Min Kim
Lymphoplasmacyte-rich meningioma (LPRM) is an extremely rare variant of meningioma, and a small percentage of LPRM may be associated with IgG4-related disease. To date, the coexistence of 2 rare meningioma variants consisting of LPRM and angiomatous meningioma within one neoplasm has not been reported in the literature. A 56-year-old woman presented with episodes of frequent and severe epistaxis that began 4 months ago. Initial magnetic resonance imaging showed localized, heterogeneous, and intermediate to high signal lesion at the odontoid process and clivus...
August 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28859705/immunoglobulin-g4-sinusitis-in-association-with-aspirin-exacerbated-respiratory-disease
#19
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
#20
Larissa Derzko-Dzulynsky
PURPOSE OF REVIEW: IgG4-related disease is a relatively newly described, multiorgan, fibro-inflammatory disease with characteristic histopathology showing lymphoplasmacytic infiltration and 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...
August 30, 2017: Current Opinion in Ophthalmology
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