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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/28223204/increases-in-ige-eosinophils-and-mast-cells-can-be-used-in-diagnosis-and-to-predict-relapse-of-igg4-related-disease
#1
Emma L Culver, Ross Sadler, Adrian C Bateman, Mateusz Makuch, Tamsin Cargill, Berne Ferry, Rob Aalberse, Eleanor Barnes, Theo Rispens
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD) is characterized by increased serum levels of IgG4 and infiltration of biliary, and pancreatic, and other tissues by IgG4-positive plasma cells. We assessed the prevalence of allergy and/or atopy, serum and tissue IgE antibodies, and blood and tissue eosinophils in patients with IgG4-RD. We investigated the association between serum IgE and diagnosis and relapse of this disease. METHODS: We performed a prospective study of 48 patients with IgG4-RD, 42 patients with an increased serum level of IgG4 with other inflammatory and autoimmune conditions (disease controls), and 51 healthy individuals (healthy controls) recruited from Oxford, United Kingdom from March 2010 through March 2014, and followed for a median of 41 months (range 3-73 months)...
February 18, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28207938/comparison-of-clinical-and-pathological-features-of-lung-lesions-of-systemic-igg4-related-disease-and-idiopathic-multicentric-castleman-s-disease
#2
Yasuhiro Terasaki, Shinichro Ikushima, Shoko Matsui, Akira Hebisawa, Yasunori Ichimura, Shinyu Izumi, Masuo Ujita, Machiko Arita, Keisuke Tomii, Youko Komase, Isoko Owan, Tetsuji Kawamura, Yasuo Matsuzawa, Miho Murakami, Hiroshi Ishimoto, Hiroshi Kimura, Masashi Bando, Norihiro Nishimoto, Yoshinori Kawabata, Yuh Fukuda, Takashi Ogura
AIMS: The lung lesion (IgG4-L) of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion (iMCD-L) of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4(+) cells in lung specimens; typical extrapulmonary manifestations)...
February 16, 2017: Histopathology
https://www.readbyqxmd.com/read/28205524/interleukin-33-produced-by-m2-macrophages-and-other-immune-cells-contributes-to-th2-immune-reaction-of-igg4-related-disease
#3
Sachiko Furukawa, Masafumi Moriyama, Kensuke Miyake, Hitoshi Nakashima, Akihiko Tanaka, Takashi Maehara, Mana Iizuka-Koga, Hiroto Tsuboi, Jun-Nosuke Hayashida, Noriko Ishiguro, Masaki Yamauchi, Takayuki Sumida, Seiji Nakamura
IgG4-related disease (IgG4-RD) is characterized by elevated serum IgG4 and marked infiltration of IgG4-positive cells in multiple organs. Interleukin-33 (IL-33) is a recently described cytokine that is secreted by damaged epithelial cells, macrophages, and dendritic cells, and potently activates helper T type 2 (Th2) immune responses, which have been suggested to play a major role in IgG4 production of IgG4-RD. Here, we assessed the expression of IL-33 and related molecules in the salivary glands (SGs) of patients with IgG4-RD versus that in patients with Sjögren's syndrome (SS) and controls...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28197739/diagnosis-and-treatment-of-igg4-related-disease
#4
Terumi Kamisawa, Kazuichi Okazaki
It is critical to differentiate IgG4-related disease (IgG4-RD) from malignant tumor and similar disease of the affected organ to apply appropriate therapy and avoid unnecessary surgery. IgG4-RD is diagnosed on combination of typical radiological findings; elevation of serum IgG4 levels; histopathological findings of abundant infiltration of IgG4-positive plasma cells and lymphocytes, storiform fibrosis , and obliterative phlebitis ; association with other IgG4-related diseases; and response to steroids. Histopathological approach is particularly recommended...
February 15, 2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28183334/circulating-plasmablasts-plasma-cells-a-potential-biomarker-for-igg4-related-disease
#5
Wei Lin, Panpan Zhang, Hua Chen, Yu Chen, Hongxian Yang, Wenjie Zheng, Xuan Zhang, Fengxiao Zhang, Wen Zhang, Peter E Lipsky
BACKGROUND: Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a multisystem fibroinflammatory disease. We previously reported that a circulating cell population expressing CD19(+)CD24(-)CD38(hi) was increased in patients with IgG4-RD. In this study, we aimed to document that this cell population represented circulating plasmablasts/plasma cells, to identify the detailed phenotype and gene expression profile of these IgG4-secreting plasmablasts/plasma cells, and to determine whether this B-cell lineage subset could be a biomarker in IgG4-related disease (IgG4-RD)...
February 10, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28166682/clonally-expanded-cytotoxic-cd4-t-cells-and-the-pathogenesis-of-igg4-related-disease
#6
Hamid Mattoo, John H Stone, Shiv Pillai
IgG4-related disease (IgG4-RD) is a systemic condition of unknown cause characterized by highly fibrotic lesions, with dense lymphoplasmacytic infiltrates containing a preponderance of IgG4-expressing plasma cells. CD4(+) T cells and B cells constitute the major inflammatory cell populations in IgG4-RD lesions. IgG4-RD patients with active, untreated disease show a marked expansion of plasmablasts in the circulation. Although the therapeutic depletion of B cells suggests a role for these cells in the disease, a direct role for B cells or IgG4 in the pathogenesis of IgG4-RD is yet to be demonstrated...
February 2017: Autoimmunity
https://www.readbyqxmd.com/read/28165852/current-approach-to-the-diagnosis-of-igg4-related-disease-combination-of-comprehensive-diagnostic-and-organ-specific-criteria
#7
Hisanori Umehara, Kazuichi Okazaki, Takuji Nakamura, Tomomi Satoh-Nakamura, Akio Nakajima, Mitsuhiro Kawano, Tsuneyo Mimori, Tsutomu Chiba
IgG4-related disease (IgG4-RD) is a fascinating clinical entity proposed by Japanese investigators, and includes a wide variety of diseases, formerly diagnosed as Mikulicz's disease (MD), autoimmune pancreatitis (AIP), interstitial nephritis, prostatitis, retroperitoneal fibrosis, etc. Although all clinicians in every field of medicine may encounter this new disease, a unifying diagnostic criteria has not been established. In 2011, the Japanese IgG4 team, organized by the Ministry of Health, Labor and Welfare (MHLW) of Japan, published comprehensive diagnostic criteria for IgG4-RD...
February 6, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28158996/igg4-positive-cell-infiltration-in-various-cardiovascular-disorders-results-from-histopathological-analysis-of-surgical-samples
#8
Ryoto Hourai, Satomi Kasashima, Koichi Sohmiya, Yohei Yamauchi, Hideki Ozawa, Yoshinobu Hirose, Yasuhiro Ogino, Takahiro Katsumata, Masahiro Daimon, Shu-Ichi Fujita, Masaaki Hoshiga, Nobukazu Ishizaka
BACKGROUND: The diagnosis of Immunoglobulin G4 (IgG4)-related disease (IgG4-RD), in general, depends on serum IgG4 concentrations and histopathological findings; therefore, diagnosis of IgG4-RD in cardiovascular organs/tissues is often difficult owing to the risk of tissue sampling. METHODS: Prevalence of IgG4-positive lymphoplasmacytic infiltration in 103 consecutive cardiovascular surgical samples from 98 patients with various cardiovascular diseases was analyzed immunohistochemically...
February 3, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28149653/igg4-related-disease-presenting-as-isolated-scleritis
#9
Eran Berkowitz, Ella Arnon, Alona Yaakobi, Yuval Cohen, Beatrice Tiosano
A rare case of IgG4-related disease (IgG4-RD) manifesting as nodular scleritis is presented in a 20-year-old female. Patient complained of left eye pain and redness for one week. Ocular examination together with ancillary testing led to the diagnosis of nodular scleritis. Since the patient did not show apparent improvement after one week of systemic steroidal treatment, she underwent a biopsy of the affected area revealing histopathological characteristics of IgG4-RD. Long-term treatment with corticosteroids and a steroid-sparing agent (methotrexate) led to significant improvement in signs and symptoms...
2017: Case Reports in Ophthalmological Medicine
https://www.readbyqxmd.com/read/28124667/igg4-related-disease-a-disease-we-probably-often-overlook
#10
I M Celis, R L Kriekaart, R P Aliredjo, E G van Lochem, M J D L van der Vorst, R Hassing
IgG4-related disease (IgG4-RD) is an increasingly recognised entity characterised by tumefied lesions that can affect multiple organs. Awareness of IgG4-RD is important, as it has been shown to mimic other diseases and may result in irreversible organ damage if not treated. If suspected, immunostaining for IgG4-positive plasma cells is essential for diagnosis and revision of old biopsies may be necessary.
January 2017: Netherlands Journal of Medicine
https://www.readbyqxmd.com/read/28101487/immunoglobulin-g4-related-thyroid-diseases
#11
REVIEW
Dulani Kottahachchi, Duncan J Topliss
Immunoglobulin G4-related disease (IgG4-RD) is a new disease category involving many organ systems, including the endocrine system in general and the thyroid in particular. Since an initial association was made between hypothyroidism and autoimmune (IgG4-related) pancreatitis, more forms of IgG4-related thyroid disease (IgG4-RTD) have been recognized. Four subcategories of IgG4-RTD have so far been identified: Riedel thyroiditis (RT), fibrosing variant of Hashimoto thyroiditis (FVHT), IgG4-related Hashimoto thyroiditis, and Graves disease with elevated IgG4 levels...
December 2016: European Thyroid Journal
https://www.readbyqxmd.com/read/28091934/roles-of-plasmablasts-and-b-cells-in-igg4-related-disease-implications-for-therapy-and-early-treatment-outcomes
#12
Marco Lanzillotta, Emanuel Della-Torre, John H Stone
High serum IgG4 concentrations are a striking feature of many patients with IgG4-related disease (IgG4-RD). Blood levels of IgG4 often reach ten, twenty, and even thirty or more times higher than the upper limit of normal. Under the proper clinical circumstances, the finding of an elevated serum IgG4 concentration serves as a useful biomarker for the diagnosis of this condition. This serum IgG4 elevation quickly called attention to the possibility of therapies targeting cells of the B lymphocyte lineage. In addition, a greater understanding of the cellular mechanisms that underpin IgG4-RD has identified peripheral blood plasmablasts as a promising biomarker for this disease...
January 7, 2017: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28091932/current-concept-of-igg4-related-disease
#13
Kazuichi Okazaki, Hisanori Umehara
IgG4-related disease (IgG4-RD) is a fibroinflammatory disease of unknown etiology, which is characterized by a tendency to form tumefactive lesions, increased serum levels of IgG4, and massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis. Patients with IgG4-RD have frequently multiorgan involvements such as the pancreas, biliary tree, salivary glands, periorbital tissues, kidneys, lungs, lymph nodes, and retroperitoneum. IgG4-RD mainly affects middle-aged to elderly men except for involvement in lachrymal and salivary glands, so-called Mikulicz's disease...
December 28, 2016: Current Topics in Microbiology and Immunology
https://www.readbyqxmd.com/read/28079853/solely-lung-involved-igg4-related-disease-a-case-report-and-review-of-the-literature
#14
Xiao-Qin Zhang, Guo-Ping Chen, Sheng-Chang Wu, Sa Yu, Hong Wang, Xuan-Yi Chen, Zhuo-Chao Ren
By analyzing the clinical data of 1 case of IgG4-related lung disease(IgG4-RLD) and the review of literature, the author investigated the clinical characteristics of IgG4-RLD. IgG4-RLD is a rare disease characterized by significant elevation of serum IgG4 and infiltration of a large number of IgG4+ plasma cells. The clinical manifestations of the disease were nonspecific, and the imaging features were mixed with several types. The disease can only be involved in the lung, but also multiple organ involvement...
December 23, 2016: Sarcoidosis, Vasculitis, and Diffuse Lung Diseases: Official Journal of WASOG
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#15
REVIEW
Giovanni Maria Rossi, Rossana Rocco, Eugenia Accorsi Buttini, Chiara Marvisi, Augusto Vaglio
Retroperitoneal fibrosis (RPF) is a rare disease characterised by fibrous tissue proliferation in the retroperitoneum, with encasement of the ureters and large vessels of the abdomen as the most destructive of potentially severe complications. It can either be idiopathic, or secondary to infections, malignancies, or the use of certain drugs. The idiopathic form accounts for approximately 75% of the cases, and is usually responsive to immunosuppressive therapy. In recent years, the emergence of a new clinical entity, IgG4-related disease (IgG4-RD), shed light on many fibro-inflammatory disorders once thought to be separate clinical entities, although frequently associated in the so-called multifocal fibrosclerosis...
January 9, 2017: Internal and Emergency Medicine
https://www.readbyqxmd.com/read/28049999/overlap-of-post-obstructive-diuresis-and-unmasked-diabetes-insipidus-in-a-case-of-igg4-related-retroperitoneal-fibrosis-and-tuberoinfundibular-hypophysitis-a-case-report-and-review-of-the-literature
#16
REVIEW
Midori Sasaki Yatabe, Kimio Watanabe, Yoshimitsu Hayashi, Junichi Yatabe, Satoshi Morimoto, Atsuhiro Ichihara, Masaaki Nakayama, Tsuyoshi Watanabe
The clinical picture of IgG4-related disease (IgG4-RD) is diverse because various organs can be affected. We describe the case of a 56-year-old man with acute renal failure and tuberoinfundibular hypophysitis due to IgG4-RD. Steroid therapy lowered the serum IgG4 level and ameliorated renal dysfunction, bilateral hydronephrosis and retroperitoneal fibrosis. However, polyuria from post-obstructive diuresis and unmasked central diabetes insipidus ensued. The patient's polyuria continued despite the administration of a therapeutic dose of glucocorticoid; the patient's pituitary swelling and anterior pituitary dysfunction were partially ameliorated...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28005278/igg4-related-disease-and-lymphocyte-variant-hypereosinophilic-syndrome-a-comparative-case-series
#17
Mollie N Carruthers, Sujin Park, Graham W Slack, Bakul I Dalal, Brian F Skinnider, David F Schaeffer, Jan P Dutz, Joanna K Law, Fergal Donnellan, Vladimir Marquez, Michael Seidman, Patrick C Wong, Andre Mattman, Luke Y C Chen
OBJECTIVE: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia, and elevated immunoglobulins/IgE. METHOD: Comparative case series of 31 patients with IgG4-RD and 13 patients with L-HES. RESULTS: Peripheral blood eosinophilia was present in eight of 31 patients with IgG4-RD compared to 13 of 13 patients with L-HES (median eosinophils 0...
December 22, 2016: European Journal of Haematology
https://www.readbyqxmd.com/read/27994271/retroperitoneal-fibrosis-the-state-of-the-art
#18
REVIEW
Marta Runowska, Dominik Majewski, Mariusz Puszczewicz
Retroperitoneal fibrosis (RPF) is a rare disease, hallmarked by inflammation and deposition of fibrous tissue around the abdominal aorta. This process may spread contiguously and involve adjacent structures, leading to many complications, among which the most frequent and most severe is ureteral obstruction. The condition usually has idiopathic origin (idiopathic retroperitoneal fibrosis - IRF), but can also develop secondarily to a number of factors. The etiology of the disease remains unclear. Current research suggests that about half of the cases of IRF may be a symptom of a recently discovered, clinically heterogeneous immunoglobulin G4-related disease (IgG4-RD)...
2016: Reumatologia
https://www.readbyqxmd.com/read/27989497/dacryoadenitis-associated-with-igg4-related-disease-in-an-afro-colombian-adolescent
#19
Gabriel Sebastián Díaz-Ramírez, Luis Fernando Medina-Quintero, Alexander Salinas-César, Andrés Felipe Zea-Vera
IgG4-related disease (IgG4-RD) is a recently recognized clinical condition with multiple aspects not yet elucidated. It is characterized by a fibrous inflammatory process that involves multiple organs and clinical, serological and histopathological findings, which represent a major challenge for the clinician. Classically described as an expansive tumor lesion with storiform fibrosis, lymphoplasmacytic infiltration (IgG4-positive) and elevated serum IgG4. Clinical features are variable, and pancreatic as well as extrapancreatic involvement has been reported, more frequently in Asian men over 50 years and rarely described in black people...
December 15, 2016: Reumatología Clinica
https://www.readbyqxmd.com/read/27988433/igg4-related-disease-evidence-from-six-recent-cohorts
#20
REVIEW
Fernando Martínez-Valle, Andreu Fernández-Codina, Iago Pinal-Fernández, Olimpia Orozco-Gálvez, Miquel Vilardell-Tarrés
IgG4-related disease (IgG4-RD) is a rare autoimmune fibrosing disorder. In this review we aim to describe and compare the characteristics of the six largest IgG4-RD cohorts, since the new 2012 consensus diagnostic criteria were released. These observational studies were published between 2012 and 2015. Patients were included using the comprehensive diagnostic criteria or the 2012 consensus criteria. Results were reviewed and summarized. Most patients were middle aged men. Fibro-inflammatory masses developed in virtually all organs except the brain, with an unexplained preference for salivary glands, lymph nodes and pancreas...
February 2017: Autoimmunity Reviews
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