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IgG4

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17 papers 100 to 500 followers
By Ben Samstein Hepatobiliary and transplant surgeon
https://www.readbyqxmd.com/read/27843427/isolated-liver-hilar-infiltration-by-igg4-inflammation-mimicking-cholangiocarcinoma
#1
Laurent Bochatay, Pietro Majno, Emiliano Giostra, Jean Louis Frossard
IgG4-related disease represents a heterogeneous group of disease characterized by infiltration of various tissues by IgG4 plasmocytes. In case of liver infiltration, this condition classically mimics primary sclerosing cholangitis or multifocal cholangiocarcinoma due to inflammation that preferentially affects the intra- and extrahepatic bile duct. Diagnostic criteria have recently been reviewed in order to better define the disease and help physicians make the diagnosis. Herein, we present the case of a patient who died after liver surgery for suspected cholangiocarcinoma that finally turned out to be IgG4-associated liver disease, a condition being out of current consensual criteria...
September 2016: Case Reports in Gastroenterology
https://www.readbyqxmd.com/read/26641633/igg4-associated-cholangitis-a-mimic-of-psc
#2
Ulrich Beuers, Lowiek M Hubers, Marieke Doorenspleet, Lucas Maillette de Buy Wenniger, Paul L Klarenbeek, Kirsten Boonstra, Cyriel Ponsioen, Erik Rauws, Niek de Vries
IgG4-associated cholangitis (IAC) is an inflammatory disorder of the biliary tract representing a major manifestation of IgG4-related disease (IgG4-RD) often with elevation of serum IgG4 levels, infiltration of IgG4+ plasma cells in the affected tissue and good response to immunosuppressive treatment. Its first description may go back to 150 years ago. The clinical presentation of IAC is often misleading, mimicking other biliary diseases such as primary sclerosing cholangitis (PSC) or cholangiocarcinoma. The HISORt criteria--histopathological, imaging, and serological features (sIgG4), other organ manifestations of IgG4-RD and response to treatment--are the standard for the diagnosis of IAC...
2015: Digestive Diseases
https://www.readbyqxmd.com/read/26259410/a-case-of-autoimmune-cholangitis-misdiagnosed-for-cholangiocarcinoma-how-to-avoid-unnecessary-surgical-intervention
#3
Igor I Ignjatović, Slavko V Matić, Vladimir D Dugalić, Djordje M Knežević, Marjan T Micev, Marko D Bogdanović, Srbislav M Knežević
INTRODUCTION: Autoimmune cholangitis or immunoglobulin G4-associated cholangitis (IAC) has been recently regarded as a new clinical and histopathological entity and is a part of a complex autoimmune disorder--IgG4-related systemic disease (ISD). ISD is an autoimmune disease with multi-organic involvement, characterized with IgG4-positive plasmocytic infiltration of various tissues and organs with a consequent sclerosis, which responds well to steroid therapy. Most commonly affected organs are the pancreas (autoimmune pancreatitis, [AIP]) and the common bile duct (IAC)...
May 2015: Srpski Arhiv za Celokupno Lekarstvo
https://www.readbyqxmd.com/read/25632939/igg4-sclerosing-cholangitis-in-a-pediatric-patient
#4
Danya Rosen, Swan Thung, Shari Sheflin-Findling, Joanne Lai, Ally Rosen, Ronen Arnon, Jaime Chu
IgG4 sclerosing cholangitis (IgG4-SC) is an immune-mediated process that results in inflammation and fibrosis of the pancreatobiliary tract. Although IgG4-SC is predominantly associated with autoimmune pancreatitis, IgG4-SC as its own entity can be difficult to diagnose. Patients with IgG4-SC are typically men over the age of 60, and present clinically with obstructive jaundice, abdominal pain, and weight loss. The diagnosis of IgG4-SC may be difficult to differentiate from primary sclerosing cholangitis (PSC) or cholangiocarcinoma...
February 2015: Seminars in Liver Disease
https://www.readbyqxmd.com/read/25655558/association-between-hla-haplotypes-and-increased-serum-levels-of-igg4-in-patients-with-primary-sclerosing-cholangitis
#5
MULTICENTER STUDY
Natalie L Berntsen, Olav Klingenberg, Brian D Juran, Maria Benito de Valle, Björn Lindkvist, Konstantinos N Lazaridis, Kirsten Muri Boberg, Tom H Karlsen, Johannes Roksund Hov
Increased serum levels of IgG4 have been reported in 9%-15% of patients with primary sclerosing cholangitis (PSC); it is not clear whether this increase contributes to pathogenesis. We performed genetic analyses of the HLA complex in patients with PSC from Norway, Sweden, and from the United States. We found an association between levels of IgG4 above the upper reference limit and specific HLA haplotypes. These patients had a significantly lower frequency of the strongest PSC risk factor, HLA-B*08, than patients without increased IgG4, and significantly higher frequencies of HLA-B*07 and HLA-DRB1*15...
May 2015: Gastroenterology
https://www.readbyqxmd.com/read/25481618/igg4-related-disease
#6
REVIEW
Terumi Kamisawa, Yoh Zen, Shiv Pillai, John H Stone
IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognised as a unified entity only 10 years ago. Histopathology is the key to diagnosis. The three central pathology features of IgG4-related disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis...
April 11, 2015: Lancet
https://www.readbyqxmd.com/read/25179687/immunoglobulin-g4-related-sclerosing-cholangitis
#7
Deepak Joshi, George J Webster
No abstract text is available yet for this article.
April 2015: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/24011021/-plasma-cell-hepatitis-in-liver-allografts-identification-and-characterization-of-an-igg4-rich-cohort
#8
M Castillo-Rama, M Sebagh, E Sasatomi, P Randhawa, K Isse, A D Salgarkar, K Ruppert, A Humar, A J Demetris
Plasma cell hepatitis (PCH), also known as "de novo autoimmune" hepatitis, is an increasingly recognized, but suboptimally named and poorly understood, category of late allograft dysfunction strongly resembling autoimmune hepatitis (AIH): They share plasma-cell-rich necro-inflammatory activity on biopsy, autoantibodies and steroid responsiveness, but overlap with rejection is problematic. A retrospective study of clinical, serological, histopathological and IgG4 immunohistological features of PCH (n = 20) in liver allograft recipients, native liver AIH (n = 19) and plasma-cell-rich renal allograft rejection (n = 20) showed: (1) high frequency (44%) of HLA-DR15; (2) less female predominance (p = 0...
November 2013: American Journal of Transplantation
https://www.readbyqxmd.com/read/25155229/type-1-autoimmune-pancreatitis-and-igg4-related-sclerosing-cholangitis-is-associated-with-extrapancreatic-organ-failure-malignancy-and-mortality-in-a-prospective-uk-cohort
#9
Matthew T Huggett, E L Culver, M Kumar, J M Hurst, M Rodriguez-Justo, M H Chapman, G J Johnson, S P Pereira, R W Chapman, George J M Webster, E Barnes
OBJECTIVES: Type I autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-related SC) are now recognized as components of a multisystem IgG4-related disease (IgG4-RD). We aimed to define the clinical course and long-term outcomes in patients with AIP/IgG4-SC recruited from two large UK tertiary referral centers. METHODS: Data were collected from 115 patients identified between 2004 and 2013, and all were followed up prospectively from diagnosis for a median of 33 months (range 1-107), and evaluated for response to therapy, the development of multiorgan involvement, and malignancy...
October 2014: American Journal of Gastroenterology
https://www.readbyqxmd.com/read/22341851/the-immunobiology-and-clinical-characteristics-of-igg4-related-diseases
#10
Hiroki Takahashi, Motohisa Yamamoto, Tetsuya Tabeya, Chisako Suzuki, Yasuyoshi Naishiro, Yasuhisa Shinomura, Kohzoh Imai
Having the characteristic features of elevated serum IgG4 levels and prominent infiltration of IgG4-positive plasma cells with fibrosis in lesions, Mikulicz's disease (MD) has been recognized as an IgG4-related disease (IgG4-RD). Although incidence of autoimmune pancreatitis (AIP), one of the organ characteristics of IgG4-RD, has been internationally reported, there are only a few such reports of IgG4-related MD. The limited number of reports might be attributable to the low recognition of IgG4-related MD as a clinical entity as well as its misdiagnosis as Sjögren's syndrome (SS)...
August 2012: Journal of Autoimmunity
https://www.readbyqxmd.com/read/24651618/the-diagnostic-utility-of-serum-igg4-concentrations-in-igg4-related-disease
#11
Mollie N Carruthers, Arezou Khosroshahi, Tamara Augustin, Vikram Deshpande, John H Stone
OBJECTIVES: We evaluated the sensitivity, specificity and positive and negative predictive values of elevated serum IgG4 concentrations for the diagnosis of IgG4-RD. METHODS: Between 2001 and 2011, 190 unique patients had elevated serum IgG4 measurements. We reviewed electronic medical records to determine the indication for IgG4 measurement and underlying clinical diagnosis. Additionally, we reviewed the records of 190 other randomly selected patients from a pool of 3360 with normal results, to evaluate test characteristics of the IgG4 measurement...
January 2015: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/22717980/clinical-diagnostic-criteria-of-igg4-related-sclerosing-cholangitis-2012
#12
Hirotaka Ohara, Kazuichi Okazaki, Hirohito Tsubouchi, Kazuo Inui, Shigeyuki Kawa, Terumi Kamisawa, Susumu Tazuma, Kazushige Uchida, Kenji Hirano, Hitoshi Yoshida, Takayoshi Nishino, Shigeru B H Ko, Nobumasa Mizuno, Hideaki Hamano, Atsushi Kanno, Kenji Notohara, Osamu Hasebe, Takahiro Nakazawa, Yasuni Nakanuma, Hajime Takikawa
BACKGROUND: IgG4-sclerosing cholangitis (IgG4-SC) patients have an increased level of serum IgG4, dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall, and a good response to steroid therapy. However, it is not easy to distinguish IgG4-SC from primary sclerosing cholangitis, pancreatic cancer, and cholangiocarcinoma on the basis of cholangiographic findings alone because various cholangiographic features of IgG4-SC are similar to those of the above progressive or malignant diseases...
September 2012: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/24768756/igg4-cholangiopathy-current-concept-diagnosis-and-pathogenesis
#13
Kazuichi Okazaki, Kazushige Uchida, Masanori Koyabu, Hideaki Miyoshi, Tsukasa Ikeura, Makoto Takaoka
IgG4 related cholangiopathy, a distinctive type of cholangitis of unknown origin, is characterized by increased serum levels of IgG4, massive infiltration of IgG4-positive plasma cells with storiform fibrosis and/or obliterative phlebitis in the thickened bile duct wall, and good response to steroids. Patients with IgG4-cholangiopathy are frequently associated with autoimmune pancreatitis; IgG4-cholangiopathy is recognized as a biliary manifestation of IgG4-related disease. This condition can be diagnosed by a combination of imaging, serology, histopathology, and steroid responsiveness; however, cholangiographic features are often difficult to differentiate from primary sclerosing cholangitis, pancreatic cancer, or cholangiocarcinoma...
September 2014: Journal of Hepatology
https://www.readbyqxmd.com/read/19399359/incidence-clinical-presentation-and-pathological-features-of-benign-sclerosing-cholangitis-of-unknown-origin-masquerading-as-biliary-carcinoma
#14
COMPARATIVE STUDY
Takeo Fujita, Motohiro Kojima, Naoto Gotohda, Shinichiro Takahashi, Toshio Nakagohri, Masaru Konishi, Atsushi Ochiai, Taira Kinoshita
BACKGROUND: Benign obstructions of the liver hilum are occasionally encountered in surgically resected cases. Some of these cases are pathologically classified as benign sclerosing cholangitis and are not clearly categorized. This study aims to elucidate the clinicopathological features of benign sclerosing cholangitis of unknown origin. METHODS: Patients who had undergone surgery of the proximal bile duct from 1993 to 2008 on suspicion of proximal bile carcinoma were evaluated, then their clinical presentation, histopathological and immunohistochemical findings were retrospectively analyzed...
March 2010: Journal of Hepato-biliary-pancreatic Sciences
https://www.readbyqxmd.com/read/22580936/cholangiocarcinoma-or-igg4-associated-cholangitis-how-feasible-it-is-to-avoid-unnecessary-surgical-interventions
#15
Dimitrios Lytras, Evangelos Kalaitzakis, George J M Webster, Charles J Imber, Zahir Amin, Manuel Rodriguez-Justo, Stephen P Pereira, Steven W M Olde Damink, Massimo Malagoʼ
OBJECTIVE: To evaluate the experience of a tertiary hepatopancreaticobiliary (HPB) center in the diagnostic approach and management of patients with suspicion of cholangiocarcinoma (CCa), focusing on excluding patients with IgG4-associated cholangitis (IAC) from unnecessary major surgical interventions. METHODS: Between January 2008 and September 2010, a total number of 152 patients with suspicion of CCa underwent evaluation through a HPB multidisciplinary team meeting...
December 2012: Annals of Surgery
https://www.readbyqxmd.com/read/24341121/igg4-related-sclerosing-disease-an-emerging-entity-frequently-misdiagnosed
#16
REVIEW
Roberta Mazzucchelli, Stefano Racchini, Francesca Barbisan, Andrea B Galosi, Sara Giorgini
IgG4-related sclerosing disease, a multiorgan system disease that has been identified in the last 10 years, is a fibroinflammatory condition with a marked propensity to manifest itself as mass forming lesions characterized by three main histological features (sclerosis, obliterative phlebitis and lymphoplasmacytic infiltrate) and by the presence of abundant IgG4+ plasma cells, frequent elevation of serum IgG4 and a dramatic initial response to steroid therapy. The aim of this mini-review is to increase the capacity to identify the characteristic features of IgG4-related sclerosing disease in specific organs and in two newly proposed entities (urethral caruncle and paratesticular fibrous pseudotumor) using biopsy specimens and methods of counting IgG4...
August 2013: Analytical and Quantitative Cytopathology and Histopathology
https://www.readbyqxmd.com/read/23540501/igg4-associated-cholangitis
#17
REVIEW
Marina G Silveira
IgG4-associated cholangitis is the hepatobiliary manifestation of a recently characterized inflammatory systemic disease, associated with increased IgG4 serum levels and IgG4-positive lymphoplasmacytic infiltration. Often, patients present with obstructive jaundice, and imaging reveals stenoses of the extrahepatic or intrahepatic bile ducts, often in association with parenchymal pancreatic findings and irregularities of the pancreatic duct. The histologic findings include lymphoplasmacytic infiltrates, on occasion resulting in tumefactive lesions (which can mimic malignancy), obliterative phlebitis, and fibrotic changes...
May 2013: Clinics in Liver Disease
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