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IgG4 sclerosing disease

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https://www.readbyqxmd.com/read/29963328/benign-sclerosing-and-fibrosing-conditions-of-the-abdomen-and-their-potential-mimics
#1
REVIEW
Joseph H Mullineux, Catalin V Ivan, Jay Pancholi, Ratan Verma, Arumugam Rajesh, Sadhna Verma, James A Stephenson
The process of abnormal reparative or reactive processes in the abdominal cavity, can lead to sclerosis and fibrous deposition. The relatively recent discovery of an IgG4 subgroup of immune mediated sclerosing disease 1,2 has thrown some light on the pathophysiology of these conditions. Firstly, our pictorial review aims to describe imaging findings to enhance the general radiologist's recognition and interpretation of this varied group of benign sclerotic and fibrotic abdominal processes. Secondly, along with the imaging findings, we bring into discussion the potential mimics of these pathologic processes to minimise interpretational errors...
2018: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/29924455/igg4-related-disease-review-of-the-histopathologic-features-differential-diagnosis-and-therapeutic-approach
#2
REVIEW
Jacob R Bledsoe, Emanuel Della-Torre, Lucrezia Rovati, Vikram Deshpande
Immunoglobulin G4-related disease (IgG4-RD) is an uncommon disorder that demonstrates characteristic clinicopathologic features including sclerosing lesions with storiform fibrosis, increased IgG4+ plasma cells with an increased IgG4+/IgG+ plasma cell ratio, obliterative phlebitis, and often an increased serum IgG4 level. This review summarizes the characteristic histopathologic and clinical features of IgG4-RD with detailed discussion of the histopathologic characteristics of the most commonly involved anatomic sites...
June 2018: APMIS: Acta Pathologica, Microbiologica, et Immunologica Scandinavica
https://www.readbyqxmd.com/read/29805707/differential-diagnostic-roles-of-the-serum-ca19-9-total-bilirubin-tbil-and-the-ratio-of-ca19-9-to-tbil-for-benign-and-malignant
#3
Wei Liu, Qiaofei Liu, Wenqin Wang, Penghui Wang, Jieming Chen, Tao Hong, Ning Zhang, Binglu Li, Qiang Qu, Xiaodong He
Background: Obstructive jaundice is one of the most common symptoms which can be caused by both malignant and benign hepato-biliary-pancreatic diseases. The differences and the differential diagnostic roles of the serum CA19-9, total bilirubin (TBIL) and especially, the ratio of CA19-9 to TBIL in these patients have not been well elucidated. This study compared the differences and the differential diagnostic roles of the increase-folds of the serum CA19-9, TBIL and the ratio of increase-folds of CA19-9 to increase-folds of TBIL in 508 cases of malignant (MOJ) and benign (BOJ) obstructive jaundice patients...
2018: Journal of Cancer
https://www.readbyqxmd.com/read/29774578/the-immunobiology-of-rankl-and-myeloid-derived-suppressor-cell-activation-in-igg4-related-sclerosing-cholangitis
#4
Min Lian, Qixia Wang, Xiang Jiang, Jun Zhang, Yiran Wei, Yanmei Li, Bo Li, Weihua Chen, Haiyan Zhang, Qi Miao, Yanshen Peng, Xiao Xiao, Li Sheng, Weici Zhang, Jingyuan Fang, Ruqi Tang, M Eric Gershwin, Xiong Ma
The primary function of myeloid derived suppressor cells (MDSCs) is reflected in their immune modulatory role in several immune-mediated diseases. In IgG4-related disease (IgG4-RD), it has been hypothesized that there are selective regulatory defects that lead to a Th2 bias immune response. Herein we have taken advantage of a large cohort of patients with IgG4-related sclerosing cholangitis (IgG4-SC), the most common extra-pancreatic involvement of IgG4-RD, as well as controls consisting of primary sclerosing cholangitis (PSC), autoimmune hepatitis (AIH) and healthy volunteers, to study MDSC...
May 18, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/29767269/-igg4-associated-cholangitis-clinical-presentation-of-an-overlooked-disease-entity
#5
REVIEW
T Herta, J Verheij, U Beuers
IgG4-associated cholangitis (IAC) is the hepatobiliary manifestation of immunoglobulin G4-related disease, which is an immune-mediated fibroinflammatory systemic disorder characterised by often elevated IgG4 serum levels and typical histopathological findings in affected tissues. IAC is frequently (>90%) accompanied by autoimmune pancreatitis type 1 (AIP), which is the pancreatic manifestation of immunoglobulin G4-related disease. In 80-85% of the cases patients with IAC are male, above 50 years of age and present with jaundice and weight loss...
June 2018: Der Internist
https://www.readbyqxmd.com/read/29766732/-remission-of-the-disease-associated-related-with-immunoglobulin-igg4-accompanied-by-multiple-lymphadenopathy-after-treatment-with-rituximab-and-dexamethasone-a-case-report
#6
Zdeněk Adam, Zita Chovancová, Markéta Nová, Pavel Fabian, Zdeněk Řehák, Renata Koukalová, Marek Slávik, Luděk Pour, Marta Krejčí, Aleš Čermák, Zdeněk Král, Jiří Mayer
A disease associated with immunoglobulin IgG4 is a rare unit with very variable symptoms. We describe the course and treatment of the disease in a patient who presented with multiple lymphadenopathy and infiltrates in the area of the retroperitoneum and pelvis and signs of chronic sclerosing pancreatitis. The disease was clinically manifested by a significant loss of weight, but also by a loss of perception of taste and smell. The diagnosis was made based on a high amount of IgG4 expressing plasma cells in the sampled tissue and an increased concentration of immunoglobulins of type IgG and mainly subclass IG4...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29742724/a-case-report-of-immunoglobulin-g4-related-sclerosing-cholangitis-with-multiple-relapse
#7
Xiaoqin Dong, Na Huo, Zhao Wu, Guiqiang Wang, He Wang, Hong Zhao
RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital with neoplasms of the bile duct and pancreas at first visit in December 2011...
May 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29738364/a-newly-recognized-histologic-pattern-of-igg4-related-lymphadenopathy-expanding-the-morphologic-spectrum
#8
Ying-Ren Chen, Yi-Ju Chen, Ming-Chung Wang, L Jeffrey Medeiros, Kung-Chao Chang
Immunoglobulin (Ig)G4-related sclerosing disease is a fibroinflammatory disorder characterized by tumor-forming lesions at multiple anatomic sites and by increased serum levels of IgG4. IgG4-related lymphadenopathy, defined as lymphadenopathy developing in patients with IgG4-related sclerosing disease, is known to manifest in 5 histologic patterns: (1) multicentric Castleman disease-like; (2) reactive follicular hyperplasia; (3) interfollicular plasmacytosis with immunoblasts; (4) progressive transformation of germinal centers-like; and (5) inflammatory pseudotumor-like...
July 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/29707074/hepatobiliary-manifestations-and-complications-in-inflammatory-bowel-disease-a-review
#9
REVIEW
Fotios S Fousekis, Vasileios I Theopistos, Konstantinos H Katsanos, Epameinondas V Tsianos, Dimitrios K Christodoulou
Liver and biliary track diseases are common extraintestinal manifestations of inflammatory bowel disease (IBD), reported both in Crohn's disease and ulcerative colitis, and may occur at any time during the natural course of the disease. Their etiology is mainly related to pathophysiological changes induced by IBD, and secondary, due to drugs used in IBD. Fatty liver is considered as the most frequent hepatobiliary manifestation in IBD, while primary sclerosing cholangitis (PSC) is the most correlated hepatobiliary disorder and is more prevalent in patients with ulcerative colitis...
April 2018: Gastroenterology Research
https://www.readbyqxmd.com/read/29619414/hilar-cholangiocarcinoma-associated-with-immunoglobulin-g4-positive-plasma-cells-and-elevated-serum-immunoglobulin-g4-levels
#10
Nabeel Azeem, Veeral Ajmera, Bilal Hameed, Neil Mehta
Immunoglobulin G4 (IgG4)-related disease is a fibroinflammatory systemic disorder with multiorgan involvement. Proximal bile duct involvement results in IgG4-related sclerosing cholangitis, which is characterized by a lymphoplasmacytic infiltrate with abundant IgG4-positive plasma cells and fibrosis. Differentiating between cholangiocarcinoma and IgG4-sclerosing cholangitis can present a diagnostic dilemma. We describe an unusual presentation of a hepatic mass meeting multiple criteria for IgG4-sclerosing cholangitis but was ultimately found to be cholangiocarcinoma...
April 2018: Hepatology Communications
https://www.readbyqxmd.com/read/29562380/igg4-related-disease-of-the-hepatobiliary-tract-2-case-reports-and-review-of-the-literature
#11
REVIEW
J Spapen, A Reekmans, B Berghmans, S Debeuckelaere, P Buydens, L Trappeniers, B Van den Bossche, I Colle
IgG4-related disease is a rare inflammatory disorder that may mimic many infectious, malignant, and autoimmune conditions. The biliary tract is frequently involved, but hepatic lesions are rarely seen. Diagnosis is often delayed due to the absence of specific clinical and radiological signs, and the lack of an accurate diagnostic marker. Differential diagnosis includes cholangiocarcinoma, primary sclerosing cholangitis and intrinsic or metastatic liver disease. Corticosteroids are the cornerstone of therapy but treatment has not been standardized and relapse is common...
January 2018: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/29549357/igg4-associated-cholangitis-in-patients-resected-for-presumed-perihilar-cholangiocarcinoma-a-30-year-tertiary-care-experience
#12
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...
May 2018: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/29489505/is-sclerosing-angiomatoid-nodular-transformation-an-igg4-associated-sclerosing-disease
#13
Yan-Wen Jin, Wei Gao, Fu-Yu Li, Nan-Sheng Cheng
No abstract text is available yet for this article.
February 27, 2018: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/29467115/the-pancreatic-and-extrapancreatic-manifestations-of-igg4-related-disease
#14
Jaspreet Singh Sangha Brar, Saurav Gupta, Salahudeen M Haja Mohideen, Lishya Liauw, Narayan Lath
The association between immunoglobulin IgG4 and autoimmune pancreatitis was first shown in 2001. Since then many previously established fibrosclerotic diseases demonstrating synchronous or metachronous multiorgan involvement have been included within the ambit of IgG4-related disease. Diagnostic criteria have been proposed involving 1) serum IgG4 level elevated beyond 135 mg/dL, 2) IgG4+ to IgG+ plasma cell ratio > 40% and >10 IgG4+ cells per high power field of biopsy sample and 3) a constellation of imaging features which involve a variety of organ systems...
March 2018: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
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
#15
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/29429186/-igg4-related-sclerosing-disease-with-initial-ear-symptom-a-case-report
#16
M Hu, W Wang, Y Cheng
No abstract text is available yet for this article.
February 7, 2018: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29428100/clinical-analysis-of-liver-transplantation-in-autoimmune-liver-diseases
#17
Cheng-Peng Zhong, Zhi-Feng Xi, Qiang Xia
BACKGROUND: Autoimmune liver diseases (ALDs) consist of autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis and overlap syndromes. Patients with these diseases may gradually progress to end-stage liver diseases and need liver transplantation. The present study aimed to explore the prognosis of patients with ALDs after liver transplantation. METHODS: The clinical data of 80 patients with ALD (24 cases of AIH, 35 of PBC, 15 of PSC and 6 of AIH-PBC overlap syndromes) who underwent liver transplantation in Renji Hospital, Shanghai Jiao Tong University School of Medicine from June 2004 to September 2016 were collected retrospectively...
February 2018: Hepatobiliary & Pancreatic Diseases International: HBPD INT
https://www.readbyqxmd.com/read/29415331/immunoglobulin-g4-related-sclerosing-oesophagitis-in-a-9-year-old-girl
#18
Lucie Bienfait, Nathalie Nagy, Imad El Moussaoui, Pierre Lingier, Issam El Nakadi, Pieter Demetter, Laurine Verset
No abstract text is available yet for this article.
July 2018: Histopathology
https://www.readbyqxmd.com/read/29394821/-a-case-of-an-igg4-related-inflammatory-pseudotumor-of-the-liver-showing-enlargement-that-was-difficult-to-differentiate-from-hepatic-cancer
#19
Kei Adachi, Kazuhiko Hashimoto, Ryoji Nonaka, Jeong-Ho Moon, Yujiro Fujie, Shoichiro Fujita, Keiko Kojima, Jun Hanai, Shingi Imaoka, Tadashi Ohnishi
A 60-year-old man was admitted for a liver mass(S3), which rapidly increased in size during intraductal papillary mucinous neoplasm(IPMN)follow-up. Although EOB-MRIwas performed, the mass could not be accurately diagnosed as hepatic cancer. Thus, we performed a lateral segmentectomy. In the resected specimen, a solid tumor mass was clearly bound in segment 3 of the liver. Since histopathology revealed no malignant cells and many IgG4-positive cells, we confirmed the diagnosis as IgG4-related inflammatory pseudotumor of the liver...
November 2017: Gan to Kagaku Ryoho. Cancer & Chemotherapy
https://www.readbyqxmd.com/read/29332510/localized-autoimmune-pancreatitis-mimicking-pancreatic-cancer-case-report-and-literature-review
#20
Wen-Ling Hsu, Shu-Min Chang, Pei-Yin Wu, Chin-Chuan Chang
Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels...
April 2018: Journal of International Medical Research
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