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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28821195/atypical-igg4-plasmacytic-proliferations-and-lymphomas-characterization-of-11-cases
#1
Jacob R Bledsoe, Zachary S Wallace, Vikram Deshpande, Joshua R Richter, Jason Klapman, Andrew Cowan, John H Stone, Judith A Ferry
Objectives: To report the clinicopathologic features of monotypic immunoglobulin G4+ (IgG4+) lymphoid and plasmacytic proliferations. Methods: Cases were identified from the pathology files. Pathology and clinical materials were reviewed. Results: Eleven cases of monotypic IgG4+ proliferations were identified at nodal, orbital, or salivary sites. Six cases (three men, three women; age, 57-94 years) met criteria for lymphoma or plasma cell neoplasia...
September 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28803167/igg4-related-disease-a-new-etiology-underlying-diffuse-intracranial-dilating-vasculopathy
#2
Evan S Marlin, Davis Dornbos, Daniel S Ikeda, Norman L Lehman, Ciarán J Powers
BACKGROUND: Diffuse intracranial aneurysmal vasculopathy is a rare condition, previously described in patients with human immunodeficiency virus (HIV) infection. IgG4 related disease (IgG4-RD) is a recognized inflammatory disease of systemic organs, leading to fibrosis of connective tissues. It has also been linked to inflammatory dilating aortic aneurysms, coronary vascular disease, hypophysitis, orbital pseudotumor and pachymeningitis. It has not yet been described as a cause of diffuse intracranial dilating vasculopathy...
August 9, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28800014/large-tumefactive-igg4-related-disease-histologic-cytologic-and-immunohistochemical-features-of-a-very-unusual-case
#3
Israh Akhtar, Veena Shenoy, Majid Khan, Ali G Saad
Immunoglobulin G4-related disease (IgG4-RD) is a regional or systemic multiorgan lymphoplasmacytic inflammatory disease of unknown etiology. It has been described in numerous organs and anatomic locations. Review of the literature shows that when the disease involves the retroperitoneum it causes retroperitoneal fibrosis. Tumefactive IgG4-RD of the retroperitoneum has not been previously reported. In this report, we describe the first case of a large retroperitoneal tumefactive IgG4-RD along with its histologic, cytologic, and immunohistochemical characteristics...
August 9, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#4
REVIEW
David C Trampert, Lowiek M Hubers, Stan F J van de Graaf, Ulrich Beuers
The pathophysiology of immunoglobulin G4-related disease (IgG4-RD) and its most common manifestations, IgG4-associated (sclerosing) cholangitis and autoimmune pancreatitis, remains largely unknown, but IgG4 is presumably involved. IgG4 is a promiscuous antibody, which could be directly pathogenic, fulfill a protective role, or could just be a fortuitous marker of an aberrant inflammatory response. IgG4 antibodies possess exclusive structural and functional characteristics suggesting anti-inflammatory and tolerance-inducing effects...
August 3, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#5
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28768471/unsuspected-human-immunodeficiency-virus-infection-presenting-as-immunoglobulin-g4-related-lymphadenopathy-a-case-report
#6
Hsing-Tse Yu, Chen-Hsiang Lee, Shun-Chen Huang, Shan-Fu Yu
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is an immune-mediated condition characterized by infiltration of the involved organs by IgG4-bearing plasma cells. The prevalence of autoimmune diseases, associated with or occurring in patients with human immunodeficiency virus (HIV) infection, has been increasing. We describe a 58-year-old man with an undiagnosed HIV infection, which presented as chronic cervical lymphadenopathy with an elevated serum IgG4 and a very high IgE. Histologically, lymph nodes showed expanded sinusoids and burnt-out germinal centers with increased plasmacytic infiltration and collagen fiber deposition...
January 1, 2017: International Journal of STD & AIDS
https://www.readbyqxmd.com/read/28765476/annexin-a11-is-targeted-by-igg4-and-igg1-autoantibodies-in-igg4-related-disease
#7
Lowiek M Hubers, Harmjan Vos, Alex R Schuurman, Robin Erken, Ronald P Oude Elferink, Boudewijn Burgering, Stan F J van de Graaf, Ulrich Beuers
OBJECTIVE: Immunoglobulin G4-related disease (IgG4-RD) is a multiorgan immune-mediated disease that predominantly affects the biliary tract (IgG4-associated cholangitis, IAC) and pancreas (autoimmune pancreatitis, AIP). We recently identified highly expanded IgG4+ B-cell receptor clones in blood and affected tissues of patients with IAC/AIP suggestive of specific (auto)antigenic stimuli involved in initiating and/or maintaining the inflammatory response. This study aimed to identify (auto)antigen(s) that are responsible for the clonal expansion of IgG4+ B cells in IgG4-RD...
August 1, 2017: Gut
https://www.readbyqxmd.com/read/28747608/igg4-related-disease-mechanistic-insights-from-both-clinical-and-immunologic-understanding-of-this-condition
#8
Takashi Maehara
  IgG4-related disease (IgG4-RD) is a chronic inflammatory disease characterized by tumescent lesions with characteristic storiform fibrosis, obliterative phlebitis and a marked lymphoplasmacytic infiltrate that includes a large number of IgG4 positive plasma cells. It's widely accepted that rituximab-mediated B cell depletion therapy is effective for this disease. Important mechanistic insights correlated with the pathogenesis of IgG4-RD have been gradually disclosed from studies of patients treated by B cell depletion...
2017: Nihon Rinshō Men'eki Gakkai Kaishi, Japanese Journal of Clinical Immunology
https://www.readbyqxmd.com/read/28736430/a-case-of-type-1-autoimmune-pancreatitis-aip-a-form-of-igg4-related-disease-igg4-rd
#9
Mounira El Euch, Souha Hddad, Madiha Mahfoudhi, Hela Maktouf, Fethi Ben Hamida, Fatima Jaziri, Khaoula Ben Abdelghani, Sami Turki, Taïeb Ben Abdallah
BACKGROUND Type 1 autoimmune pancreatitis (AIP), also known as lymphoplasmacytic sclerosing pancreatitis (LPSP), is a rare cause of chronic pancreatitis, characterized by a fibro-inflammatory process. However, patients with AIP may have a good response to corticosteroid therapy. We describe a Tunisian patient with AIP that was confirmed to be an IgG4-related disease (IgG4-RD). CASE REPORT We describe a case of a 70-year-old man who was admitted to hospital for obstructive jaundice and abdominal pain. Serum liver function tests were abnormal and upper abdominal computed tomography (CT) imaging showed diffuse pancreatic swelling and strictures of the main pancreatic duct without any focal lesion...
July 24, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28735232/immunoglobulin-g4-related-mastitis-a-case-report
#10
Ee Syn Tan, Brendon Friesen, Seow Foong Loh, Jane Fox
INTRODUCTION: IgG4-related mastitis (IgG4-RM) is exceedingly rare with only ten cases reported in the literature to date. Organs that are affected with IgG4-related disease (IgG4-RD) all share the same histopathological hallmarks consisting of dense lymphocytic infiltration, storiform fibrosis and obliterative phlebitis. PRESENTATION OF CASE: This case report highlights a case of IgG4-RM found incidentally in a 52-year-old woman during a routine breast screen and it explores the current literature about IgG4-RM and IgG4-RD...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28733656/efficacy-of-cyclophosphamide-treatment-for-immunoglobulin-g4-related-disease-with-addition-of-glucocorticoids
#11
Fei Yunyun, Chen Yu, Zhang Panpan, Chen Hua, Wu Di, Zhao Lidan, Peng Linyi, Wang Li, Wu Qingjun, Zhang Xuan, Zhao Yan, Zeng Xiaofeng, Zhang Fengchun, Zhang Wen
Aim to evaluate the efficacy and safety of glucocorticoid monotherapy vs combination therapy of cyclophosphamide (CYC) for IgG4 related disease (IgG4-RD). 102 newly diagnosed IgG4-RD patients were enrolled and assigned to 2 groups: Group I was prednisone monotherapy (0.5-1.0 mg/kg.d, tapered gradually) and Group II was glucocorticoid and CYC (50-100 mg per day). Patients were assessed at different periods. Primary end point was relapse rate; secondary end points included response, remission rate and adverse effects...
July 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28710703/igg4-related-disease-involving-polyserous-effusions-with-elevated-serum-interleukin-6-levels-a-case-report-and-literature-review
#12
Xiang Tong, Min Bai, Weiya Wang, Qingbing Han, Panwen Tian, Hong Fan
Immunoglobulin G4-related disease (IgG4-RD) is a recently described immune-mediated fibroinflammatory disease with a characteristic histopathologic appearance that can affect various organs. We report a 43-year-old Chinese female patient with IgG4-RD involving polyserous effusions with reports of worsening exertional dyspnea for 3 months. Laboratory blood tests revealed that serum interleukin (IL)-6, carbohydrate antigens (CA-199 and CA-125), and alpha-fetoprotein levels were significantly increased, but serum IgG4 levels were normal...
August 2017: Immunologic Research
https://www.readbyqxmd.com/read/28701054/diagnostic-and-treatment-workup-for-igg4-related-disease
#13
Mary Abraham, Arezou Khosroshahi
IgG4-Related Disease is a newly recognized condition which is increasingly diagnosed by practitioners due to improvement in clinical awareness. Men and women have been found to be affected by this disease in various organs, more commonly with involvement of the salivary and lacrimal glands as well as pancreas and liver. Areas covered: The diagnosis and management of this condition remain challenging as biomarkers and therapies are being investigated. Hallmark features on histology are still the gold standard for confirmation of diagnosis, whereas serum IgG4 level has been shown to be neither necessary nor sufficient for the diagnosis...
September 2017: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/28681251/igg4-related-disease-a-complex-under-diagnosed-clinical-entity
#14
REVIEW
Sujani Yadlapati, Elijah Verheyen, Petros Efthimiou
IgG4-related disease (IgG4-RD) encompasses a spectrum of complex fibro-inflammatory disorders which are often under diagnosed due to unfamiliarity by clinicians. A challenging multitude of clinical manifestations makes the diagnosis cumbersome. The primary clinical feature in IgG4-RD entails a tumor-like presentation coupled with tissue-destructive lesions. Histopathological findings include lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. These findings, in combination with elevated serum immunoglobulin G4 levels, are diagnostic in the setting of single- or multi-organ involvement...
July 5, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28639844/diagnostic-sensitivity-of-cutoff-values-of-igg4-positive-plasma-cell-number-and-igg4-positive-cd138-positive-cell-ratio-in-typical-multiple-lesions-of-patients-with-igg4-related-disease
#15
Ichiro Mizushima, Kazunori Yamada, Kenichi Harada, Shoko Matsui, Takako Saeki, Satoru Kondo, Masayuki Takahira, Yuko Waseda, Yasuhito Hamaguchi, Hiroshi Fujii, Masakazu Yamagishi, Mitsuhiro Kawano
OBJECTIVES: This study aimed to investigate the diagnostic sensitivity of the cutoff values of IgG4-positive plasma cell (PC) number and IgG4-positive/CD138-positive cell ratio proposed by the International consensus statement (ICS) on the pathology of IgG4-related disease (IgG4-RD) in typical multiple lesions of patients with IgG4-RD. METHODS: We evaluated IgG4-positive PC number and IgG4-positive/CD138-positive cell ratio in 39 samples from 18 IgG4-RD patients having more than two typical lesions of IgG4-RD...
June 22, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28621822/cdna-microarray-analysis-identifies-nr4a2-as-a-novel-molecule-involved-in-the-pathogenesis-of-sj%C3%A3-gren-s-syndrome
#16
H Takahashi, H Tsuboi, H Asashima, T Hirota, Y Kondo, M Moriyama, I Matsumoto, S Nakamura, T Sumida
To examine genes expressed specifically in labial salivary glands (LSGs) of patients with Sjögren's syndrome (SS) in comparison with those of patients with immunoglobulin (Ig)G4-related disease (IgG4-RD), and to identify the genes involved in the pathogenesis of SS. Gene expression in LSGs of SS patients, IgG4-RD patients and healthy controls (HC) was analysed by cDNA microarray. Quantitative polymerase chain reaction (qPCR) was used to validate the up-regulation of differentially expressed genes (DEGs) in SS...
June 16, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28614225/immunoglobulin-g4-related-disease-preceded-by-lung-involvement-a-case-report
#17
Miki Abo, Hazuki Takato, Satoshi Watanabe, Kazumasa Kase, Tamami Sakai, Hayato Koba, Johsuke Hara, Takashi Sone, Hideharu Kimura, Kazuo Kasahara
RATIONALE: Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition involving various organs and vessels including the pancreas, bile duct, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, meninges, and aorta. Recently, some cases of IgG4-RD have been reported, in which only pulmonary lesions were present. It is not known whether IgG4-RD can be diagnosed on the basis of pulmonary lesions only, because increases in serum IgG4 levels and infiltration of IgG4-positive plasma cells into the lung tissue also occur in other inflammatory conditions...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28614220/rituximab-was-used-to-treat-recurrent-igg4-related-hypophysitis-with-ophthalmopathy-as-the-initial-presentation-a-case-report-and-literature-review
#18
REVIEW
Wei-Jun Gu, Qian Zhang, Jian Zhu, Jie Li, Shi-Hui Wei, Yi-Ming Mu
RATIONALE: Immunoglobulin G4 (IgG4)-related hypophysitis is a type of IgG4-related disease (IgG4-RD), which is characterized by plasma cells infiltration in the pituitary causing functional changes and (or) space-occupying effect in the pituitary. IgG4-related hypophysitis is sensitive to hormone therapy in most patients, but recurrence is very likely. PATIENT CONCERNS: Here, we report a 57-year-old male patient with bilateral eye redness as the initial presentation...
June 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28607901/igg4-related-disease-igg4-rd-presenting-as-a-mass-in-the-carotid-triangle-masquerading-paraganglioma
#19
T Ramadass, V Balaji, S K J Sheba, K S Vali Ahmed, Raees Abdurahiman
IgG4-related disease (IgG4-RD) which is a protein disorder presented as a mass in the right carotid triangle in a 30 year male patient, who underwent battery of tests is described. The radiologist opined the mass as paraganglioma and the spindle shaped character of the mass also suggested neurogenic tumor in differential diagnosis. Reference to vascular surgeon also opined the same, and adviced for incisional biopsy. Histopathology report suggested IgG4-RD and immunochemistry confirmed the final diagnosis...
June 2017: Indian Journal of Otolaryngology and Head and Neck Surgery
https://www.readbyqxmd.com/read/28596203/surgical-management-of-isolated-mesenteric-autoimmune-disease-addressing-the-spectrum-of-igg4-related-disease-and-sclerosing-mesenteritis
#20
Alissa Greenbaum, Nour Yadak, Steven Perez, Ashwani Rajput
IgG4-related disease (IgG4-RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG4 and tissue IgG4 levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG4-RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG4-RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass...
June 8, 2017: BMJ Case Reports
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