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IgG4- related diseases ( IgG4-RD )

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https://www.readbyqxmd.com/read/29629225/igg4-related-disease-in-the-stomach-which-was-confused-with-gastrointestinal-stromal-tumor-gist-two-case-reports-and-review-of-the-literature
#1
Ho Seok Seo, Yoon Ju Jung, Cho Hyun Park, Kyo Young Song, Eun Sun Jung
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disorder characterized by specific pathological findings and elevated serum IgG4 level. IgG4-RD in the stomach is rare, and occasionally diagnosed as gastric subepithelial tumor (SET) by endoscopy or computed tomography scan. Two female patients in the age group of 40-50 years were diagnosed with 4 cm sized gastric SET. One underwent laparoscopic gastric wedge resection. Another one had a history of subtotal gastrectomy for early gastric cancer and idiopathic thrombocytopenic purpura with oral steroids administration...
March 2018: Journal of Gastric Cancer
https://www.readbyqxmd.com/read/29622113/imaging-of-sj%C3%A3-gren-syndrome-and-immunoglobulin-g4-related-disease-of-the-salivary-glands
#2
REVIEW
Akifumi Fujita
The salivary glands are commonly affected in systemic autoimmune disease and diseases of unknown pathogenesis. Sjögren syndrome (SjS) can be affected by other systemic diseases. Immunoglobulin G4-related disease (IgG4-RD) commonly affects salivary glands. Imaging findings are usually nonspecific; however, radiologists should be familiar with the manifestations to avoid diagnostic delay. Findings of early-stage SjS are difficult to identify on routine computed tomography or MR imaging. Chronic SjS can be diagnosed from MR imaging and sialographic findings...
May 2018: Neuroimaging Clinics of North America
https://www.readbyqxmd.com/read/29584907/comment-on-igg4-related-disease-presenting-with-raised-serum-igg2-real-timeline-of-igg4-rd
#3
Andre Mattman, Luke Y C Chen, Grace van der Gugten, Alex Chin, Mollie Carruthers, Mari L DeMarco, Daniel T Holmes
No abstract text is available yet for this article.
March 23, 2018: Rheumatology
https://www.readbyqxmd.com/read/29584900/comment-on-igg4-related-disease-presenting-with-raised-serum-igg2-real-timeline-of-igg4-rd-reply
#4
Lisa Dunkley, Hardeep S Mudhar
No abstract text is available yet for this article.
March 23, 2018: Rheumatology
https://www.readbyqxmd.com/read/29584535/intracranial-hypertension-related-to-cerebral-venous-thrombosis-and-acute-ischemic-stroke-with-micro-infarcts-associated-with-igg4-related-disease
#5
Esme Ekizoglu, Oguzhan Coban, Cagri Ulukan, Duygu Gezen Ak, Erdinç Dursun, Erdem Tuzun, Betul Baykan
IgG4-related systemic disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells, affecting multiple organs. This report describes a case who was diagnosed with IgG4-RD, having cerebral venous thrombosis and a subsequent acute ischemic stroke of undetermined cause. The 47-year-old woman presented with headache, visual disturbance and eyelid swelling and two years later she was admitted with acute attacks of mild left lower limb sensory-motor monoparesis. Indirect immunohistochemistry assay showed elevated level of IgG4, labeling neurons of the central nervous system, suggesting an immunological process possibly affecting vascular structures...
March 27, 2018: International Journal of Neuroscience
https://www.readbyqxmd.com/read/29549357/igg4-associated-cholangitis-in-patients-resected-for-presumed-perihilar-cholangiocarcinoma-a-30-year-tertiary-care-experience
#6
Eva Roos, Lowiek M Hubers, Robert J S Coelen, Marieke E Doorenspleet, Niek de Vries, Joanne Verheij, Ulrich Beuers, Thomas M van Gulik
BACKGROUND: Distinguishing perihilar cholangiocarcinoma (PHC) from benign forms of sclerosing cholangitis affecting the hilar bile ducts is challenging, since histological confirmation of PHC is difficult to obtain and accurate non-invasive diagnostic tests are not available. IgG4-associated cholangitis (IAC), an imitator of PHC, may present with clinical and radiographical signs of PHC. IAC can be accurately diagnosed with a novel qPCR test. The aim of this study was to investigate the incidence and long-term activity of IAC in patients resected for PHC in a single tertiary center over a period of 30 years...
March 16, 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29526692/rituximab-maintenance-therapy-reduces-rate-of-relapse-of-pancreaticobiliary-immunoglobulin-g4-related-disease
#7
Shounak Majumder, Sonmoon Mohapatra, Ryan J Lennon, Guilherme Piovezani Ramos, Neil Postier, Ferga Gleeson, Michael J Levy, Randall K Pearson, Bret T Petersen, Santhi S Vege, Suresh T Chari, Mark Topazian, Thomas E Witzig
BACKGROUND & AIMS: Immunoglobulin G4-related disease (IgG4-RD), a multi-organ fibro-inflammatory syndrome, typically responds to steroids. However, some cases are steroid resistant, and pancreaticobiliary IgG4-RD commonly relapses after steroid withdrawal. Rituximab induces remission of IgG4-RD, but the need for and safety of maintenance rituximab treatment are unknown. We compared outcomes of patients with pancreaticobiliary IgG4-RD treated with or without maintenance rituximab therapy...
March 8, 2018: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29526046/transbronchial-lung-biopsy-for-the-diagnosis-of-igg4-related-lung-disease
#8
Kyoko Otani, Dai Inoue, Tomoo Itoh, Yoh Zen
AIMS: The diagnosis of IgG4-related disease (IgG4-RD) requires a multidisciplinary approach, in which histology plays an important role. Although a diagnosis was previously established using surgically resected specimens, there is increasing clinical demand to diagnose this systemic condition by biopsies. The present study aimed to elucidate how useful transbronchial lung biopsies (TBLBs) are for this diagnostic purpose. METHODS AND RESULTS: The study cohort consisted of 20 consecutive patients diagnosed with IgG4-RD in other organs who underwent TBLBs for potential pulmonary involvement...
March 11, 2018: Histopathology
https://www.readbyqxmd.com/read/29512140/autoimmune-pancreatitis-mouse-model
#9
Ken Kamata, Tomohiro Watanabe, Kosuke Minaga, Warren Strober, Masatoshi Kudo
Autoimmune pancreatitis (AIP) is a chronic fibro-inflammatory disorder of the pancreas. However, extensive clinico-pathological analyses have revealed that AIP is, in reality, a pancreatic manifestation of a newly described systemic disease known as IgG4-related disease (IgG4-RD). IgG4-RD is characterized by enhanced local and systemic IgG4 antibody (Ab) responses as well as inflammation involving multiple organs, including the pancreas, bile ducts, and salivary glands. Although mice lack the IgG4 Ab subtype, autoimmune-prone MRL/Mp mice treated with repeated injection with polyinosinic-polycytidylic acid (poly (I:C)) provide an experimental model of AIP...
February 21, 2018: Current Protocols in Immunology
https://www.readbyqxmd.com/read/29502226/infrequent-organ-involvement-of-igg4-related-diseases-a-literature-review
#10
Jie Chang, Wen Zhang
IgG4-related disease (IgG4-RD) is a chronic and systemic disease that can involve multiple organs. The most commonly involved organs include the salivary glands, orbital adnexal structures, paranasal sinus, thyroid, lungs, breasts, aorta, pancreas, biliary ducts, kidneys, retroperitoneum, lymph nodes, prostate, pituitary, and endocranium. Due to increased disease research, several new site-specific nuances of IgG4-RD have been described. The authors have reviewed the recent literature and briefly summarize the infrequent organ involvement of IgG4-RD...
March 3, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29499100/a-cd8%C3%AE-subset-of-cd4-slamf7-cytotoxic-t-cells-is-expanded-in-patients-with-igg4-related-disease-and-decreases-following-glucocorticoid-treatment
#11
Emanuel Della-Torre, Emanuele Bozzalla-Cassione, Clara Sciorati, Eliana Ruggiero, Marco Lanzillotta, Silvia Bonfiglio, Hamid Mattoo, Cory A Perugino, Enrica Bozzolo, Lucrezia Rovati, Paolo Giorgio Arcidiacono, Gianpaolo Balzano, Dejan Lazarevic, Chiara Bonini, Massimo Falconi, John H Stone, Lorenzo Dagna, Shiv Pillai, Angelo A Manfredi
OBJECTIVE: An unconventional population of CD4+ SLAMF7+ cytotoxic TEM cells (CD4+ CTLs) has been linked causally to IgG4-related disease (IgG4-RD). Glucocorticoids represent the first line therapeutic approach in patients with IgG4-RD but their mechanism of action in this specific condition remains unknown. Here we describe the impact of glucocorticoids on CD4+ CTLs in IgG4-RD. METHODS: CD8α, granzyme A, perforin, and SLAMF7 expression within the effector/memory compartment of CD45RO (TEM ) and CD45RA (TEMRA ) CD4+ T cells was quantified by flow cytometry in 18 active IgG4-RD patients at baseline and after 6 months of glucocorticoid treatment...
March 2, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29465558/analysis-of-clinical-characteristics-and-treatment-of-immunoglobulin-g4-associated-cholangitis-a-retrospective-cohort-study-of-39-iac-patients
#12
Jianchun Xiao, Peiran Xu, Binglu Li, Tao Hong, Wei Liu, Xiaodong He, Chaoji Zheng, Yupei Zhao
Immunoglobulin (Ig)G4-associated cholangitis (IAC) is one of the common organ manifestations of IgG4-related systemic disease (ISD). IAC and autoimmune pancreatitis (AIP) may mimic sclerosing cholangitis, cholangiocarcinoma, or pancreatic carcinoma. Diagnosis is based on a combination of clinical, biochemical, radiological, and histological findings.To study the clinical presentation of and treatment strategy for IAC, we reviewed clinical, serologic, and imaging characteristics, as well as treatment response, in 39 patients with IAC...
February 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29465374/no-specific-imaging-pattern-can-help-differentiate-igg4-related-disease-from-idiopathic-retroperitoneal-fibrosis-18-histologically-proven-cases
#13
Alexandra Forestier, David Buob, Tristan Mirault, Philippe Puech, Viviane Gnemmi, David Launay, Eric Hachulla, Pierre-Yves Hatron, Marc Lambert
OBJECTIVES: Retroperitoneal fibrosis (RPF) is a rare disease, with unknown aetiology (idiopathic RPF: iRPF) in two-thirds of cases. A subset of iRPF may be a manifestation of IgG4-related disease (IgG4-RD). Thus, recognition of IgG4-RD-RPF is crucial to optimise patient's care with iRPF. The current study aimed to examine imaging specific patterns, which could help differentiate between IgG4-RD-RPF and iRPF, and thus skip performing biopsies. METHODS: This analysis included patients with iRPF and a retroperitoneal biopsy at the Lille University Hospital, France...
February 14, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29465360/serum-soluble-interleukin-2-receptor-is-a-useful-biomarker-for-disease-activity-but-not-for-differential-diagnosis-in-igg4-related-disease-and-primary-sj%C3%A3-gren-s-syndrome-adults-from-a-defined-population
#14
Mitsuhiro Akiyama, Takanori Sasaki, Yuko Kaneko, Hidekata Yasuoka, Katsuya Suzuki, Kunihiro Yamaoka, Tsutomu Takeuchi
OBJECTIVES: To identify biomarkers for disease activity in IgG4-related disease (IgG4-RD) and primary Sjögren's syndrome (pSS). METHODS: Forty-three consecutive treatment-naïve patients with IgG4-RD, 62 patients with pSS, and 5 patients with sicca syndrome were enrolled. IgG4-RD and pSS disease activity was assessed based on the IgG4-RD responder index (IgG4-RD RI) and EULAR Sjögren's Syndrome Disease Activity Index (ES- SDAI), respectively. The associations of biomarkers with disease activity were examined...
February 15, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29460239/clinical-and-pathophysiological-aspects-of-type-1-autoimmune-pancreatitis
#15
REVIEW
Kazushige Uchida, Kazuichi Okazaki
In 1995, Yoshida and colleagues proposed the concept of "autoimmune pancreatitis" (AIP), which has recently been recognized as a new pancreatic inflammatory disease. Recent studies have suggested the existence of two subtypes of AIP: type 1, which involves immunoglobulin G4 (IgG4) and is the pancreatic manifestation of IgG4-related disease (IgG4-RD); and type 2, which is characterized by granulocytic epithelial lesions. Type 2 AIP is thought to be rare in Japan. Type 1 AIP is characterized by increased serum IgG4 concentrations, lymphoplasmacytic infiltrations, storiform fibrosis, and obliterative phlebitis...
February 19, 2018: Journal of Gastroenterology
https://www.readbyqxmd.com/read/29460058/the-immunologic-paradoxes-of-igg4-related-disease
#16
REVIEW
Xiao Xiao, Min Lian, Weici Zhang, M Eric Gershwin, Xiong Ma
IgG4-related disease (IgG4-RD), which usually occurs in middle-aged and elderly men, is a newly recognized fibroinflammatory condition characterized by swelling and sclerosis of involved organs, increased IgG4-positive plasma cell infiltration in lesions, and elevated IgG4 concentration in serum. Despite growing interest in the research, the pathophysiological mechanism remains elusive. Most IgG4-RD patients respond well to steroid therapy initially, but recurrent and refractory cases are common, especially in advanced fibrotic stage...
February 19, 2018: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/29457382/an-international-multi-specialty-validation-study-of-the-igg4-related-disease-responder-index
#17
Zachary S Wallace, Arezou Khosroshahi, Mollie D Carruthers, Cory A Perugino, Hyon Choi, Corrado Campochiaro, Emma L Culver, Frank Cortazar, Emanuel Della-Torre, Mikael Ebbo, Ana Fernandes, Luca Frulloni, Philip Hart, Omer Karadag, Shigeyuki Kawa, Mitsuhiro Kawano, Myung-Hwan Kim, Marco Lanzillotta, Shoko Matsui, Kazuichi Okazaki, Jay H Ryu, Takako Saeki, Nicolas Schleinitz, Paula Tanasa, Hisanori Umehara, George Webster, Wen Zhang, John H Stone
OBJECTIVE: IgG4-related disease (IgG4-RD) can cause fibro-inflammatory lesions in nearly any organ, leading to organ dysfunction and failure. The IgG4-RD Responder Index (RI) was developed to help investigators assess the efficacy of treatment in a structured manner. We sought to validate the RI in a multi-national investigation. METHODS: The RI guides investigators through assessments of disease activity and damage in 25 domains, incorporating higher weights for disease manifestations that require treatment urgently or that worsen despite treatment...
February 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29456677/concurrent-eosinophilia-and-igg4-related-disease-in-a-child-a-case-report-and-review-of-the-literature
#18
Can Chen, Kuang Chen, Xilian Huang, Kaile Wang, Shenxian Qian
The current study presents the case of a 9-year-old Chinese boy who presented with eosinophilia and elevated serum levels of immunoglobulin G4 (IgG4). A bone marrow puncture identified an elevated eosinophil rate of 23% (normal range, <5%), which indicated eosinophilia. However, gene analysis, fluorescent in situ hybridization and other examinations, including bone marrow aspiration, blood routine, auto-antibody tests and parasitic and allergens screening, contradicted a diagnosis of secondary or clonal eosinophilia...
March 2018: Experimental and Therapeutic Medicine
https://www.readbyqxmd.com/read/29452298/treatment-and-outcomes-in-patients-with-igg4-related-disease-using-the-igg4-responder-index
#19
Andreu Fernández-Codina, Blanca Pinilla, Iago Pinal-Fernández, Cristina López, Guadalupe Fraile-Rodríguez, Eva Fonseca-Aizpuru, Iago Carballo, Pilar Brito-Zerón, Carlos Feijóo-Massó, Miguel López-Dupla, Maria Cinta Cid, Fernando Martínez-Valle
BACKGROUND: IgG4-related disease (IgG4-RD) is an autoimmune disease triggering an inflammatory cascade that leads to fibrosis. Outcome measures are limited and treatment options remain underexplored. OBJECTIVES: To assess the variation of the IgG4 responder index (IgG4-RI) in a cohort of IgG4-RD patients and to explore their treatments and outcomes. METHODS: We studied the clinical phenotype, severity of the disease and response to treatment in an ambispective multicenter cohort study including 14 different hospitals in Spain...
February 13, 2018: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/29434730/clinical-and-pathological-characteristics-of-igg4-related-interstitial-lung-disease
#20
Xiaoting Lv, Feng Gao, Qicai Liu, Sheng Zhang, Zhihua Huang, Yongping Zhu, Haiyang Zong, Quwen Li, Sanyan Li
IgG4-related interstitial lung disease (IgG4-RILD), which is characterized by increased IgG4 levels, IgG4+ plasma cell infiltration and irregular whorled fibrosis, is a recently described lung disorder that belongs to the group of systemic fibroinflammatory IgG4-related diseases (IgG4-RD). The aim of the present study was to improve the current knowledge regarding the specific clinical and histopathological characteristics of IgG4-RILD and to investigate its underlying immune mechanism in vivo . A total of 7 patients newly diagnosed with IgG4-RILD were enrolled in the present study (4 men and 3 women; mean age, 57 years; range, 29-71 years)...
February 2018: Experimental and Therapeutic Medicine
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