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IgG4 related disease, international consensus

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
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
Hyun Woo Lee, Sung-Hoon Moon, Myung-Hwan Kim, Dong Hui Cho, Jae Hyuck Jun, Kwangwoo Nam, Tae Jun Song, Do Hyun Park, Sang Soo Lee, Dong-Wan Seo, Sung Koo Lee
BACKGROUND: Type 1 autoimmune pancreatitis (AIP), as a pancreatic manifestation of IgG4-related disease, shows a favorable prognosis in the short term. However, disease relapse is common in long-term follow-up, despite a successful initial treatment response. This study aimed to identify the predictors of relapse and long-term outcomes in patients with type 1 AIP. METHODS: Patients with more than 2 years of follow-up who met the International Consensus Diagnostic Criteria for type 1 AIP were included...
January 23, 2018: Journal of Gastroenterology
Álvaro Bellido-Caparó, Jorge Espinoza-Ríos, Víctor Aguilar, Carlos García, José Luis Pinto, Martin Tagle Arróspide, Alejandro Bussalleu Rivera
Autoimmune pancreatitis type 1 (AIP) is the pancreatic manifestation of IgG4-related disease. The most frequent presentation of AIP is with obstructive jaundice. For definite diagnosis of type 1 Autoimmune pancreatitis international consensus diagnosis criteria (ICDC) for AIP are used. ICDC criteria include pancreatic parenchymal imaging, ductal imaging, serology, other organ involvement, histology, and response to steroid. We report a 52-years-old woman with rheumatoid arthritis without treatment presented with two months of abdominal pain in up-right quadrant with moderate intensity...
July 2017: Revista de Gastroenterología del Perú: órgano Oficial de la Sociedad de Gastroenterología del Perú
Ichiro Mizushima, Kazunori Yamada, Kenichi Harada, Shoko Matsui, Takako Saeki, Satoru Kondo, Masayuki Takahira, Yuko Waseda, Yasuhito Hamaguchi, Hiroshi Fujii, Masakazu Yamagishi, Mitsuhiro Kawano
OBJECTIVES: This study aimed to investigate the diagnostic sensitivity of the cutoff values of IgG4-positive plasma cell (PC) number and IgG4-positive/CD138-positive cell ratio proposed by the International consensus statement (ICS) on the pathology of IgG4-related disease (IgG4-RD) in typical multiple lesions of patients with IgG4-RD. METHODS: We evaluated IgG4-positive PC number and IgG4-positive/CD138-positive cell ratio in 39 samples from 18 IgG4-RD patients having more than two typical lesions of IgG4-RD...
March 2018: Modern Rheumatology
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
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
Shigeyuki Kawa
Although now considered to be a member of the systemic entity of immunoglobulin G4- (IgG4-) related disease, IgG4-related pancreatitis is generally referred to as type 1 autoimmune pancreatitis (AIP). Type 1 AIP was established based on a pathological background of lymphoplasmacytic sclerosing pancreatitis, high serum IgG4 concentration, and abundant IgG4-bearing plasma cell infiltration. The characteristic clinical features of type 1 AIP, such as elderly male preponderance, obstructive jaundice, and mass-forming lesions in the pancreas, often mimic those of pancreatic cancer...
August 2016: Seminars in Liver Disease
S Detlefsen, G Klöppel
At an international consensus conference in 2011, multifocal chronic fibrosing inflammatory processes, which are associated with elevated IgG4 serum levels and/or tissue infiltration with IgG4 positive plasma cells, were recognized as a distinct disease entity called IgG4-related disease (IgG4-RD). As IgG4-RD responds well to steroid treatment but imitates a tumor in many organs, particularly in the pancreas, a biopsy for confirmation of the diagnosis is often warranted. The histological criteria for IgG4-RD as defined in 2011 are based on the following main features: 1) dense lymphoplasmacytic infiltrate, 2) storiform fibrosis and 3) obliterative phlebitis...
September 2016: Zeitschrift Für Rheumatologie
George J Webster
Autoimmune pancreatitis (AIP) was recognized as a clinical entity, at least in the West little more than 10 years ago. Since then, studies globally, and international collaboration, have led to important advances in our understanding of its clinical features, disease course, and management, although the aetiopathogenesis of this curious disease remains to be fully elucidated. Types 1 and 2 AIP have been described, of which type 1 is the commonest form, and best defined. International consensus now recognizes it as one of the many clinical manifestations of IgG4-related disease, and is now termed IgG4-related pancreatitis (IgG4-RP)...
2016: Digestive Diseases
Oscar Ardila-Suarez, Andy Abril, José A Gómez-Puerta
IgG4-related disease is the term used to refer to a condition characterized by a lymphoplasmacytic infiltrate, fibrosis and an increased number of IgG4+ cells present in tissue, in most cases, with an elevated serum IgG4 level. This disease frequently affects the pancreas, salivary glands and lymph nodes, but can involve almost any tissue. Its etiology and the exact role of the different inflammatory cells in the damage to the target organ is still unclear. As yet, there is no international consensus about diagnostic criteria for the disease, but there are important advances in its treatment and in the quest to achieve remission...
May 2017: Reumatología Clinica
Ayodeji Oluwarotimi Omiyale
Autoimmune pancreatitis (AIP) is a rare, distinct and increasingly recognized form of pancreatitis which has autoimmune features. The international consensus diagnostic criteria (ICDC) for AIP recently described two subtypes; type 1[lymphoplasmacytic sclerosing pancreatitis (LPSP)] and type 2 [idiopathic duct-centric pancreatitis (IDCP) or AIP with granulocytic epithelial lesion (GEL)]. Type 1 is the more common form of the disease worldwide and current understanding suggests that it is a pancreatic manifestation of immunoglobulin G4-related disease (IgG4-RD)...
June 2016: Gland Surgery
T Helmberger
CLINICAL/METHODICAL ISSUE: Autoimmune pancreatitis (AIP) is a rare disease, the pathophysiological understanding of which has been greatly improved over the last years. The most common form, type 1 AIP belongs to the IgG4-related diseases and must be distinguished from type 2 AIP, which is a much rarer entity associated with chronic inflammatory bowel disease. Clinically, there is an overlap with pancreatic cancer. Imaging and further criteria, such as serological and histological parameters are utilized for a differentiation between both entities in order to select the appropriate therapy and to avoid the small but ultimately unnecessary number of pancreatectomies...
April 2016: Der Radiologe
Kazushige Uchida, Hideaki Miyoshi, Tsukasa Ikeura, Masaaki Shimatani, Makoto Takaoka, Kazuichi Okazaki
In 1995, Yoshida and colleagues proposed the concept of 'autoimmune pancreatitis' (AIP). Recently, it is accepted that the existence of two subtypes of AIP-type 1, which involves immunoglobulin G4 (IgG4) as 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. In 2011, the International Consensus Diagnostic Criteria (ICDC) for autoimmune pancreatitis was proposed. In Japan, the clinical diagnostic criteria of AIP 2011 was proposed by the Japan Pancreas Society (JPS) and the Research Committee of Intractable Diseases of the Pancreas...
February 2016: Clinical Journal of Gastroenterology
Kazuichi Okazaki, Kazushige Uchida
Before the immunoglobulin G4 (IgG4) era, autoimmune pancreatitis was proposed as a single clinical entity of autoimmune disease. In the IgG4 era, the following 2 subtypes have been proposed: type 1 is the pancreatic manifestation of IgG4-related disease and type 2 presents with granulocytic epithelial lesions. The characteristic features of type 1 are increased serum IgG4, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmacytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), other organ involvements (eg, sclerosing cholangitis, sclerosing sialadenitis, retroperitoneal fibrosis), and responsiveness to steroid...
October 2015: Pancreas
Dai Inoue, Kotaro Yoshida, Norihide Yoneda, Kumi Ozaki, Takashi Matsubara, Keiichi Nagai, Kenichirou Okumura, Fumihito Toshima, Jun Toyama, Tetsuya Minami, Osamu Matsui, Toshifumi Gabata, Yoh Zen
Immunoglobulin G4-related disease (IgG4-RD) is a recently discovered systemic condition, in which various organ manifestations are linked by a similar histological appearance. Our knowledge of this condition is still fragmented, as most studies have examined only a few dozen patients or focused on a particular organ manifestation. This study was conducted to learn the demography and patient characteristics of IgG4-RD using a large cohort. A total of 235 consecutive patients with IgG4-RD, diagnosed in 8 general hospitals in the same medical district, were identified by searching the institutions' radiology database...
April 2015: Medicine (Baltimore)
Sönke Detlefsen, Michael Bau Mortensen, Torsten Kjærulff Pless, Anne-Sofie Cribe, Ove B Schaffalitzky de Muckadell
OBJECTIVES: The aims of this study were to describe the diagnosis of autoimmune pancreatitis (AIP) in Denmark and to test the usefulness of the International Consensus Diagnostic Criteria (ICDC) on a geographically well-defined cohort. METHODS: All patients diagnosed with AIP at Odense University Hospital from 2007 to 2013 were included (n = 30; mean follow-up, 26.2 months). Data from laparoscopic or percutaneous ultrasound-guided core needle biopsy (CNB), resection specimens, endoscopic ultrasound (EUS), EUS-guided CNB, computed tomography, serum immunoglobulin G4 (IgG4), and pancreatography were retrospectively analyzed according to ICDC...
August 2015: Pancreas
A Khosroshahi, Z S Wallace, J L Crowe, T Akamizu, A Azumi, M N Carruthers, S T Chari, E Della-Torre, L Frulloni, H Goto, P A Hart, T Kamisawa, S Kawa, M Kawano, M H Kim, Y Kodama, K Kubota, M M Lerch, M Löhr, Y Masaki, S Matsui, T Mimori, S Nakamura, T Nakazawa, H Ohara, K Okazaki, J H Ryu, T Saeki, N Schleinitz, A Shimatsu, T Shimosegawa, H Takahashi, M Takahira, A Tanaka, M Topazian, H Umehara, G J Webster, T E Witzig, M Yamamoto, W Zhang, T Chiba, J H Stone
No abstract text is available yet for this article.
July 2015: Arthritis & Rheumatology
Phil A Hart, Yoh Zen, Suresh T Chari
Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that is characterized clinically by frequent presentation with obstructive jaundice, histologically by a dense lymphoplasmacytic infiltrate with fibrosis, and therapeutically by a dramatic response to corticosteroid therapy. Two distinct diseases, type 1 and type 2 AIP, share these features. However, these 2 diseases have unique pancreatic histopathologic patterns and differ significantly in their demographic profiles, clinical presentation, and natural history...
July 2015: Gastroenterology
Alexander Kleger, Thomas Seufferlein, Martin Wagner, Andrea Tannapfel, Thomas K Hoffmann, Julia Mayerle
BACKGROUND: IgG4-associated autoimmune diseases are systemic diseases affecting multiple organs of the body. Autoimmune pancreatitis, with a prevalence of 2.2 per 100,000 people, is one such disease. Because these multi-organ diseases present in highly variable ways, they were long thought just to affect individual organ systems. This only underscores the importance of familiarity with these diseases for routine clinical practice. METHODS: This review is based on pertinent articles retrieved by a selective search in PubMed, and on the published conclusions of international consensus conferences...
February 20, 2015: Deutsches Ärzteblatt International
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