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IgG4 sclerosing disease

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https://www.readbyqxmd.com/read/29332510/localized-autoimmune-pancreatitis-mimicking-pancreatic-cancer-case-report-and-literature-review
#1
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...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29313399/localized-intrahepatic-igg4-related-sclerosing-cholangitis-igg4-sc-as-an-additional-type-of-igg4-sc-a-systematic-analysis-of-12-cases
#2
Zhichao Mo, Kuvaneshan Ramen, Yunfeng Shan, Mengtao Zhou, Xiangwu Zheng, Xiuling Wu, Zhengping Yu, Xiaoke Ji, Qiyu Zhang, Qiqiang Zeng
OBJECTIVES: IgG4-related sclerosing cholangitis (IgG4-SC), a recently defined disease entity, has been classified into four types based on the stricture regions revealed by cholangiography. However, localized intrahepatic IgG4-SC is not included into the classification. This study aimed to analyze and characterize localized intrahepatic IgG4-SC and justify the inclusion of this type into the classification. METHODS: PubMed and Embase were searched for studies published from March 2001 to June 2017 reporting localized intrahepatic IgG4-SC...
January 9, 2018: Scandinavian Journal of Gastroenterology
https://www.readbyqxmd.com/read/29226107/cholangitis-diagnosis-treatment-and-prognosis
#3
REVIEW
Amir Houshang Mohammad Alizadeh
Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC)...
December 28, 2017: Journal of Clinical and Translational Hepatology
https://www.readbyqxmd.com/read/29196804/igg4-related-disease-with-emphasis-on-the-biopsy-diagnosis-of-autoimmune-pancreatitis-and-sclerosing-cholangitis
#4
REVIEW
Sönke Detlefsen, Günter Klöppel
In 2011, chronic fibroinflammatory processes occurring simultaneously or metachronously in various organs and associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4-positive plasma cells have been recognized as manifestations of a systemic disorder called IgG4-related disease (IgG4-RD). The histologic key findings are lymphoplasmacytic infiltration rich in IgG4-positive plasma cells combined with storiform fibrosis and obliterative phlebitis. Among the organs mainly affected by IgG4-RD are the pancreas and the extrahepatic bile ducts...
December 1, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29157939/lymphadenopathy-associated-with-igg4-related-disease-diagnosis-differential-diagnosis
#5
REVIEW
Mark R Wick, Dennis P O'Malley
IgG4-related sclerosing disease, which now encompasses diverse organ-related disorders with various prior eponymic designations, may also present with solitary or multifocal lymph node enlargement. This review considers the histopathologic features of IgG4 lymphadenopathy (IgG4LAD), which has been subdivided by Cheuk & Chan into 5 microscopic subtypes. Those include variants that are typified by multicentric Castleman disease (MCD)-like changes, follicular hyperplasia, interfollicular lymphoplasmacytic proliferation, progressive transformation of germinal centers, and formation of inflammatory pseudotumor (IPT)-like lesions...
November 11, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29099539/igg4-related-disease-in-thymus-a-very-rare-case-of-chronic-fibrosis-mimicking-sarcoidosis
#6
Sara Simonetti, Noelia Pérez Muñoz, Josefa López Vivancos, Lluís Sanchez Sitjes, Javier Cesar Herranz Pérez, Nelson Leal Bohorquez, José Antonio Maestre Alcacer, Inessa Koptseva de García, Miguel Ángel Carrasco García
IgG4-related disease (IgG4-RD) is a multi-organ immune-mediated chronic fibroinflammatory condition, with unclear certain etiology. It is morphologically characterized by storiform fibrosis, dense IgG4-positive lymphoplasmacytic infiltrate, and obliterative phlebitis. It was recognized as a systemic condition as recently as 2003. IgG4-RD has been described in virtually every organ, forming sclerosing masses, and often mimicking tumors. Clinically, patients present unspecific symptoms and this condition is often recognized incidentally...
October 28, 2017: Tumori
https://www.readbyqxmd.com/read/29021429/sclerosing-esophagitis-with-igg4-positive-plasma-cell-infiltration-a-case-report
#7
Shigeo Mori, Yoshiya Tahashi, Kazushige Uchida, Tsukasa Ikeura, Naoyuki Danbara, Takahiro Wakamatsu, Takeo Kusuda, Yu Takahashi, Masato Yanagawa, Mitsunobu Matsushita, Chisato Ohe, Taku Michiura, Kentaro Inoue, Masanori Kon, Kazuichi Okazaki
The patient was a 76-year-old woman who had noticed slight difficulty in swallowing in the 3 years prior to this presentation. Her dysphagia progressed while she was hospitalized following cervical cancer surgery. Esophagogastroduodenoscopy and an esophagram showed circumferential erosion and a stricture of the thoracic esophagus. Esophageal resection was performed; the resected specimens showed a stricture and wall thickening. Histologically, transmural hyperplasia, which consisted of inflammatory granulation tissue with the abundant infiltration of IgG4-positive plasma cells and lymphocytes, was observed...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28988418/clinical-utility-of-18-f-fluorodeoxyglucose-positron-emission-tomography-in-diagnosis-of-immunoglobulin-g4-related-sclerosing-sialadenitis
#8
Kenichi Takano, Ryoto Yajima, Ryuta Kamekura, Motohisa Yamamoto, Hiroki Takahashi, Naoya Yama, Masamitsu Hatakenaka, Tetsuo Himi
OBJECTIVES/HYPOTHESIS: The aim of this study was to evaluate the utility of (18) F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) for accurately diagnosing immunoglobulin G4-related sclerosing sialadenitis (IgG4-SS). STUDY DESIGN: Retrospective cohort study. METHODS: We reviewed the records of 64 patients with IgG4-SS (35 male and 29 female patients) and 10 patients with clinically suspected IgG4-SS. Pathological diagnoses of patients clinically suspected with IgG4-SS included four cases of malignant lymphoma, one case of multicentric Castleman disease, one case of Sjögren's syndrome, and four cases of sialadenitis...
October 8, 2017: Laryngoscope
https://www.readbyqxmd.com/read/28979630/sclerosing-mesenteritis-a-benign-cause-of-mesenteric-mass-lesions
#9
Diogo Carrola Gomes, Luísa Quaresma
Sclerosing mesenteritis is a rare disease of the mesentery. Associations with surgery, trauma, autoimmunity and paraneoplastic syndrome have been suggested, but most of the cases remain idiopathic. Diagnosis is often incidental, based upon the finding of a single or multiple mesenteric lesions on abdominal CT and histopathological confirmation. Optimal treatment is still controversial, but most of the cases reported have a favourable prognosis. We present a case of a 54-year-old male with long-standing abdominal pain and nausea, whose CT revealed the presence of a large mesenteric mass...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28951947/cholangiocarcinoma-and-its-mimickers-in-primary-sclerosing-cholangitis
#10
REVIEW
Jonghun John Lee, Sebastian T Schindera, Hyun-Jung Jang, Scott Fung, Tae Kyoung Kim
Cholangiocarcinoma (CCA) is the most common malignancy in primary sclerosing cholangitis (PSC). Approximately half of CCA are diagnosed within two years of initial diagnosis and often have a poor prognosis because of advanced tumor stage at the time of diagnosis. Thus, rigorous initial imaging evaluation for detecting CCA is important. CCA in PSC usually manifests as intrahepatic mass-forming or perihilar periductal-infiltrating type. Imaging diagnosis is often challenging due to pre-existing biliary strictures and heterogeneous liver...
September 26, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28922106/igg4-related-disease-a-patient-with-multiple-organ-involvement
#11
Miroslav Průcha, Lenka Sedláčková
IgG4-related diseases represent a heterogeneous group of conditions characterised by elevated serum IgG4 levels and fibrotic or sclerosing changes in the affected organs or systems accompanied by IgG4-positive plasma cells. A disease associated with IgG4 may affect virtually any organ - salivary glands, periorbital tissue, kidneys, lungs, meninges, aorta, prostate, pericardium or skin. Histopathological findings are uniform, characterised by a major lymphoplasmocytic infiltrate and the presence of IgG4-producing plasma cells, irrespective of the affected site...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28905689/igg4-related-cholangiopathy-and-its-mimickers-a-case-report-and-review-highlighting-the-importance-of-early-diagnosis
#12
Kyle Geary, Cemal Yazici, Anita Seibold, Grace Guzman
Immunoglobulin (Ig) G4 (IgG4)-related disease is a recently described clinical entity that can involve multiple organs. It is an autoimmune disorder characterized by a dense lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis. A key distinguishing factor is its dramatic response to steroid therapy. Although best described in cases of autoimmune pancreatitis, IgG4-related disease has also been implicated in patients with cholangitis and is now commonly referred to as IgG4-related cholangiopathy...
September 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28870031/-the-clinical-characteristics-of-346-patients-with-igg4-related-disease
#13
P P Zhang, J Z Zhao, M Wang, R E Feng, X W Liu, X M Lai, X J Li, J G Zeng, H J Shi, H D Zhu, W Xue, H Zhang, Y Y Chen, L Y Fei, X F Peng, F C Zeng, Y M Zhang, W Zhang
Objective: To analyze the clinical characteristics of IgG4-related disease (IgG4-RD)so as to improve the understanding of IgG4-RD in China. Methods: IgG4-RD patients were recruited from Peking Union Medical College Hospital between January 2011 and January 2016. All patients were followed-up for more than 6 months. The demographic characteristics, symptoms, organ involvements, laboratory examinations and treatment efficacy were evaluated and analyzed. Results: A total of 346 patients were finally enrolled, including 230 males (66...
September 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28816552/the-role-of-biopsy-in-lacrimal-gland-inflammation-a-clinicopathologic-study
#14
Panitee Luemsamran, Jack Rootman, Valerie A White, Nariman Nassiri, Manraj K S Heran
PURPOSE: To determine the causes of lacrimal gland inflammation based on histopathology and systemic evaluation. METHODS: This is a retrospective case series study. From the University of British Columbia Orbit Clinic between January 1976 and December 2008, we reviewed the medical records of 60 patients who presented with inflammatory features of the lacrimal gland (i.e., erythema, edema, or tenderness) in which the diagnoses were not possible clinically and on imaging alone...
August 17, 2017: Orbit
https://www.readbyqxmd.com/read/28782655/on-the-role-of-igg4-in-inflammatory-conditions-lessons-for-igg4-related-disease
#15
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 4, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28777155/a-splenic-igg4-sclerosing-angiomatoid-nodular-transformation-sant-treated-by-hemisplenectomy-a-radiologic-histochemical-and-immunohistochemical-study
#16
Raffaele Gaeta, Francescamaria Donati, Emanuele F Kauffmann, Daniela Campani
INTRODUCTION: Sclerosing angiomatoid nodular transformation (SANT) is a rare benign lesion of unknown origin for which total splenectomy is the standard treatment. CASE PRESENTATION: A 54-year-old man with a history of recurrent pancreatitis, bicuspid aortic valve, and aortic dissection underwent abdominal ultrasound, Computed tomography and magnetic resonance imaging, which revealed a 6-cm hypoechoic splenic mass diagnosed as cavernous hemangioma. Owing to his relevant past history, he was considered eligible for emisplenectomy and not for total excision, which is associated with long-term risks, especially infections...
August 2, 2017: Applied Immunohistochemistry & Molecular Morphology: AIMM
https://www.readbyqxmd.com/read/28765476/annexin-a11-is-targeted-by-igg4-and-igg1-autoantibodies-in-igg4-related-disease
#17
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/28744088/igg4-related-disease-simulating-paraneoplastic-syndrome-role-of-18-fdg-pet-ct-imaging
#18
Madhuri S Mahajan, Sudeshna Maitra, Natasha Singh, Melvika Pereira
Immunoglobulin G4 (IgG4)-related systemic disease (IgG4-RSD) is a new systemic entity associated with autoimmune pancreatitis (AIP). Other organ involvements take the form of sclerosing cholangitis, sclerosing cholecystitis, sclerosing sialadenitis, retroperitoneal fibrosis, and interstitial nephritis. Recently, lung diseases related to IgG4 have been described to occur with or without other organ involvement. These diseases include interstitial lung disease (ILD), pulmonary inflammatory pseudotumor, and lymphomatoid granulomatosis...
April 2017: Indian Journal of Radiology & Imaging
https://www.readbyqxmd.com/read/28736430/a-case-of-type-1-autoimmune-pancreatitis-aip-a-form-of-igg4-related-disease-igg4-rd
#19
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/28612666/igg4-related-sclerosing-disease-of-the-breast-in-a-male-patient
#20
Taisia Vitkovski, Galina S Marder, Dominic A Filardi, Ekta Gupta, Frank Breuer
IgG4-related sclerosing disease of the breast is a rare entity with 10 reports in the literature. We report the first case in a male patient. A 48-year-old male presented with 4-week history of palpable right upper outer quadrant breast mass associated with skin puckering. He reported a family history of breast cancer in his aunt. Ultrasound and mammography showed a spiculated 2.5-cm mass associated with skin retraction with extension to the pectoralis muscle. Ultrasound-guided core biopsy was performed. The findings were interpreted as acute and chronic inflammatory process...
June 1, 2017: International Journal of Surgical Pathology
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