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IgG4 sclerosing disease

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https://www.readbyqxmd.com/read/28328835/poor-response-of-initial-steroid-therapy-for-igg4-related-sclerosing-cholangitis-with-multiple-organs-affected
#1
Wei Liu, Weijie Chen, Xiaodong He, Qiang Qu, Tao Hong, Binglu Li
IgG4-related sclerosing cholangitis (IgG4-SC) is a rare biliary manifestation in which many other organs might be affected. The purpose of our study was to investigate the different clinical characteristics and initial steroid response between IgG4-SC patients with and without other organs affected.A series of patients with IgG4-SC in the period from January 2006 to December 2015 at our hospital were included. The pancreas and major salivary glands were screened, and the initial corticosteroid therapy was given...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28246478/eosinophilic-cholangitis-is-a-potentially-underdiagnosed-etiology-in-indeterminate-biliary-stricture
#2
Dirk Walter, Sylvia Hartmann, Eva Herrmann, Jan Peveling-Oberhag, Wolf O Bechstein, Stefan Zeuzem, Martin-Leo Hansmann, Mireen Friedrich-Rust, Jörg G Albert
AIM: To investigate presence and extent of eosinophilic cholangitis (EC) as well as IgG4-related disease in patients with indeterminate biliary stricture (IBS). METHODS: All patients with diagnosis of sclerosing cholangitis (SC) and histopathological samples such as biopsies or surgical specimens at University Hospital Frankfurt from 2005-2015 were included. Histopathological diagnoses as well as further clinical course were reviewed. Tissue samples of patients without definite diagnosis after complete diagnostic work-up were reviewed regarding presence of eosinophilic infiltration and IgG4 positive plasma cells...
February 14, 2017: World Journal of Gastroenterology: WJG
https://www.readbyqxmd.com/read/28238541/igg4-related-sclerosing-cholangitis-overlapping-with-autoimmune-hepatitis-report-of-a-case
#3
Hongyan Li, Li Sun, David R Brigstock, Lina Qi, Runping Gao
BACKGROUND: IgG4-related sclerosing cholangitis (IgG4-SC) is the biliary manifestation of IgG4-related disease (IgG4-RD) but the presence of IgG4-SC in the porta hepatis is difficult to differentiate from hilar cholangiocarcinoma (HCCA). IgG4-related autoimmune hepatitis (IgG4-related AIH) is extremely rare and it is not fully clear whether IgG4-related AIH is a hepatic manifestation of IgG4-RD or a subtype of AIH. CASE PRESENTATION: We present a rare case of a 52-year-old male who was admitted with obstructive jaundice and itchy skin...
February 10, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28225291/-a-contribution-to-the-differential-diagnostics-of-sclerosing-cholangitides
#4
Martin Blaho, Petr Dítě, Martina Bojková, Martin Rydlo, Tomáš Kupka, Pavel Svoboda, Pavel Klvaňa, Arnošt Martínek
Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28219566/immunoglobulin-g4-related-periaortitis-involving-the-aortic-arch-mimicking-a-mediastinal-tumor
#5
Min Jae Cha, Semin Chong, Yang Soo Kim, Byungjoon Park, Ja Hee Seo, Eun Sung Lee
Immunoglobulin G4 (IgG4)-related sclerosing disease is a recently proposed systemic fibroinflammatory disease entity, with periaortitis as a representative manifestation in the cardiovascular system. We present a case of IgG4-related periaortitis involving the aortic arch and proximal great vessels, mimicking an anterior mediastinal tumor on computed tomography. In patients with a soft tissue mass along the aortic wall, IgG4-related periaortitis should be included as a differential diagnosis and timely serologic investigation should be performed to prevent unnecessary surgery, because the disease shows a remarkable response to corticosteroids...
March 2017: Annals of Thoracic Surgery
https://www.readbyqxmd.com/read/28202062/isolated-igg4-related-sclerosing-cholangitis-misdiagnosed-as-malignancy-in-an-area-with-endemic-cholangiocarcinoma-a-case-report
#6
Narongsak Rungsakulkij, Pattana Sornmayura, Penampai Tannaphai
BACKGROUND: The most common cause of perihilar obstruction is cholangiocarcinoma, especially in Thailand. Benign perihilar stricture represents less than 20% of cases. IgG4-related disease and IgG4-related sclerosing cholangitis, however, have been receiving increased recognition. Isolated IgG4-related sclerosing cholangitis is less common. The preoperative diagnosis of IgG4-related sclerosing cholangitis without pancreatic involvement is very difficult because the clinical presentation and preoperative evaluation are extremely difficult to distinguish from perihilar cholangiocarcinoma...
February 15, 2017: BMC Surgery
https://www.readbyqxmd.com/read/28197205/from-pathogenesis-clinical-manifestation-and-diagnosis-to-treatment-an-overview-on-autoimmune-pancreatitis
#7
REVIEW
Ou Cai, Shiyun Tan
Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis which is autoimmune mediated. The international consensus diagnostic criteria (ICDC) 2011 proposed two types of AIP: type I is associated with histological pattern of lymphoplasmacytic sclerosing pancreatitis (LPSP), characterized by serum IgG4 elevation, whereas type 2 is named idiopathic duct-centric pancreatitis (IDCP), with granulocytic epithelial lesion (GEL) and immunoglobulin G4 (IgG4) negative. The pathogenic mechanism is unclear now; based on genetic factors, disease specific or related antigens, innate and adaptive immunity may be involved...
2017: Gastroenterology Research and Practice
https://www.readbyqxmd.com/read/28165852/current-approach-to-the-diagnosis-of-igg4-related-disease-combination-of-comprehensive-diagnostic-and-organ-specific-criteria
#8
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/28111336/clinical-features-response-to-treatment-and-outcomes-of-igg4-related-sclerosing-cholangitis
#9
Atsushi Tanaka, Susumu Tazuma, Kazuichi Okazaki, Takahiro Nakazawa, Kazuo Inui, Tsutomu Chiba, Hajime Takikawa
BACKGROUND & AIMS: Immunoglobulin G4 sclerosing cholangitis (IgG4SC) is a biliary tract manifestation of IgG4-related systemic disease. We investigated the demographics, clinical features at presentation, treatment response, and outcomes of IgG4-SC using data from a large-scale survey in Japan. METHODS: We performed a retrospective cohort study of 527 patients with IgG4-SC (436 female; median age, 66.2 years; range, 23-89 years) in Japan from 2000 to 2015. Data on patient demographics, presentation, treatment response, and outcomes were collected from questionnaires given to patients at 211 referral centers in Japan in 2015...
January 19, 2017: Clinical Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/28109714/biliary-tumors-with-pancreatic-counterparts
#10
Yasuni Nakanuma, Yoshiko Sudo
Some biliary diseases mimic pancreatic diseases pathologically as well as pathogenetically. Such diseases can be called "biliary diseases with pancreatic counterparts". Biliary intraepithelial neoplasm (BilIN), intraductal papillary neoplasm of bile ducts (IPNB), hepatobiliary mucinous cystic neoplasm (hMCN), and IgG4-inflammatory pseudotumor represent the biliary counterparts of pancreatic intraepithelial neoplasm (PanIN), intraductal papillary mucinous neoplasm of pancreas (IPMN), pancreatic MCN, and mass forming type 1 autoimmune pancreatitis (AIP), respectively...
December 20, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28105357/immunoglobulin-g4-mediated-sclerosing-cholangitis-as-a-risk-factor-for-cholangiocarcinoma-a-case-report
#11
Karin E Koopman, Elisabeth Bloemena, Geert Kazemier, Michael Klemt-Kropp
Immunoglobulin (Ig)G4-mediated disease is a systemic autoimmune disease, which occasionally presents solely as sclerosing cholangitis (SC). IgG4-mediated SC is challenging to diagnose, as it may mimic cholangiocarcinoma radiologically, and carcinoma cells may produce IgG4. The diagnosis of IgG4-mediated disease is based on histological consensus criteria and response to corticosteroids. In addition to the radiological and histological overlap between IgG4-mediated SC and cholangiocarcinoma, IgG4-mediated SC may be considered as a risk factor for the development of cholangiocarcinoma...
December 2016: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28070877/idiopathic-retroperitoneal-fibrosis-and-its-overlap-with-igg4-related-disease
#12
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/28060780/18f-fdg-pet-ct-features-of-chronic-sclerosing-sialadenitis-presenting-as-localized-igg4-related-disease
#13
Nobuo Kashiwagi, Eisuke Enoki, Chisa Hosokawa, Kenta Sakaguchi, Takamichi Murakami
Chronic sclerosing sialadenitis is a benign inflammatory condition that most commonly affects the submandibular gland in elderly individuals. It is currently known to belong to the spectrum of IgG4-related systemic diseases, which is reflected by systemic involvement on F-FDG PET/CT images. We presented a case of a 73-year-old man with histologically proven IgG4-related chronic sclerosing sialadenitis, unilateral localized form, on whole-body F-FDG PET/CT images that mimicked submandibular carcinoma with lymph node metastasis...
February 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/27977604/the-importance-of-igg4-screening-in-patients-diagnosed-with-primary-sclerosing-cholangitis-in-the-past-a-case-rediagnosed-as-igg4-sc-after-10-years
#14
Yanni Li, Lu Zhou, Xin Zhao, Wenjing Song, Nathasha Karunaratna, Bangmao Wang
RATIONALE: While primary sclerosing cholangitis (PSC) has been recognized for decades, immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) has been correctly diagnosed only in recent years. PSC and IgG4-SC show similar clinical symptoms, serologic markers, and imaging results, but the treatment strategies and prognosis of patients differ. PATIENT CONCERNS: Here, we present the case report of a patient diagnosed with PSC for 10 years and rediagnosed with IgG4-SC recently, to emphasize the importance of screening serum IgG4 levels in patients with previous diagnosis of PSC...
December 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27853829/-hepatopancreaticobiliary-diseases-in-igg4-associated-autoimmune-diseases
#15
L Grenacher
BACKGROUND: IgG4-related diseases are rare systemic multiorgan diseases and can thus affect any organ system. The incidence of diagnosis has significantly increased due to increasing awareness. OBJECTIVE: In the abdomen the hepatopancreaticobiliary system provides an essential organ system for the expression of IgG4-associated autoimmune diseases. The focus here is autoimmune pancreatitis type 1 but IgG4-associated sclerosing cholangitis and IgG4-associated hepatopathy, which can also occur in combination are less well-known...
December 2016: Der Radiologe
https://www.readbyqxmd.com/read/27843427/isolated-liver-hilar-infiltration-by-igg4-inflammation-mimicking-cholangiocarcinoma
#16
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/27797069/igg4-related-sclerosing-mesenteritis-causing-bowel-obstruction-a-case-report
#17
Atsushi Abe, Tatsuya Manabe, Nobuyoshi Takizawa, Takashi Ueki, Daisuke Yamada, Kinuko Nagayoshi, Yoshihiko Sadakari, Hayato Fujita, Shuntaro Nagai, Hidetaka Yamamoto, Yoshinao Oda, Masafumi Nakamura
Sclerosing mesenteritis (SM) is a rare inflammatory and fibrosing disease primarily involving the small-bowel mesentery. Recently, SM was reported to be closely related to IgG4-related disease (IgG4-RD). This report describes a patient with SM associated with IgG4-RD. A 77-year-old woman with a history of surgery for ectopic pregnancy and wound dehiscence presented with intestinal obstruction. Abdominal enhanced computed tomography (CT) revealed an enhanced, radially shaped, oval mass, 3 cm in diameter, with an unclear rim in the mesentery of the distal ileum, which may have involved the distal ileum...
December 2016: Surgical Case Reports
https://www.readbyqxmd.com/read/27790959/igg4-related-syndrome-another-multiorgan-disease-in-the-interest-field-of-internal-medicine
#18
Antonino Tuttolomondo, Irene Simonetta, Domenico Di Raimondo, Tiziana Di Chiara, Roberta Conigliaro, Francesca Corpora, Valerio Vassallo, Antonio Pinto
BACKGROUND: IgG4-related disease is a rare, clinical and pathologic disease entity of unknown etiology. Its main features are increased serum concentrations of IgG4 > 1,35 g/l, lymphocyte and IgG4+plasma-cell infiltration within tissues, fibrosis or sclerosis. The classical presentation of IgG4-RSD is pancreatitis which is combined with the involvement of biliary ducts in 74 percent of patients. Extrapancreatic manifestations include: abdominal or mediastinal lymphadenopathy; the involvement of salivary glands and lacrimal glands, kidneys, lung, retroperitoneum...
2016: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/27712010/hepatic-manifestations-of-inflammatory-bowel-diseases
#19
Sophie Restellini, Olivier Chazouillères, Jean-Louis Frossard
Inflammatory bowel diseases (IBD) are associated with various hepatobiliary disorders, reported both in Crohn's disease (CD) and ulcerative colitis (UC). They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions. Fatty liver is considered as the most common hepatobiliary complication in IBD whilst primary sclerosing cholangitis (PSC) the most specific one. Less frequently, IBD-associated hepatobiliary disorders include: autoimmune hepatitis/PSC overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis (PBC), hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis, and liver abscess...
October 6, 2016: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/27639563/imaging-of-autoimmune-biliary-disease
#20
Melinda J Yeh, So Yeon Kim, Kartik S Jhaveri, Spencer C Behr, Nieun Seo, Benjamin M Yeh
Autoimmune biliary diseases are poorly understood but important to recognize. Initially, autoimmune biliary diseases are asymptomatic but may lead to progressive cholestasis with associated ductopenia, portal hypertension, cirrhosis, and eventually liver failure. The three main forms of autoimmune biliary disease are primary biliary cirrhosis, primary sclerosing cholangitis, and IgG4-associated cholangitis. Although some overlap may occur between the three main autoimmune diseases of the bile ducts, each disease typically affects a distinct demographic group and requires a disease-specific diagnostic workup...
January 2017: Abdominal Radiology
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