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IgG4 related disease, international consensus

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...
June 18, 2016: 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
Hiroyuki Matsubayashi, Naomi Kakushima, Kohei Takizawa, Masaki Tanaka, Kenichiro Imai, Kinichi Hotta, Hiroyuki Ono
Autoimmune pancreatitis (AIP) is a distinct form of chronic pancreatitis that is increasingly being reported. The presentation and clinical image findings of AIP sometimes resemble those of several pancreatic malignancies, but the therapeutic strategy differs appreciably. Therefore, accurate diagnosis is necessary for cases of AIP. To date, AIP is classified into two distinct subtypes from the viewpoints of etiology, serum markers, histology, other organ involvements, and frequency of relapse: type 1 is related to IgG4 (lymphoplasmacytic sclerosing pancreatitis) and type 2 is related to a granulocytic epithelial lesion (idiopathic duct-centric chronic pancreatitis)...
November 28, 2014: World Journal of Gastroenterology: WJG
S Rasch, V Phillip, G Weirich, I Esposito, J Gaa, R M Schmid, H Algül
BACKGROUND: Autoimmune pancreatitis (AIP) was first classified as a defined disease entity in 1995. It accounts for approximately 2 % of cases of chronic pancreatitis (western world prevalence 36-41/100,000 inhabitants) and AIP is diagnosed in 2.4 % of pancreas resection specimens. OBJECTIVES: Presentation of strategies for diagnosis and treatment with focus on differentiation of AIP and pancreatic carcinoma. METHODS: Selective literature research in PubMed regarding pathogenesis, diagnosis and treatment of AIP...
October 2014: Der Internist
Derek A O'Reilly, Deep J Malde, Trish Duncan, Madhu Rao, Rafik Filobbos
Autoimmune pancreatitis (AIP) is a rare form of chronic pancreatitis, with as yet undetermined incidence and prevalence in the general population. Our understanding of it continues to evolve. In the last few years, 2 separate subtypes have been identified: type 1 AIP has been recognised as the pancreatic manifestation of a multiorgan disease, named immunoglobulin G4 (IgG4)-related disease while type 2 AIP is a pancreas specific disorder not associated with IgG4. International criteria for the diagnosis of AIP have been defined: the HISORt criteria from the Mayo clinic, the Japan consensus criteria and, most recently, the international association of pancreatology "International Consensus Diagnostic Criteria"...
May 15, 2014: World Journal of Gastrointestinal Pathophysiology
Raffaele Pezzilli, Nico Pagano
Autoimmune pancreatitis (AIP) is a recently discovered form of pancreatitis and represents one of the diseases of the pancreas which can be cured and healed medically. International consensus diagnostic criteria have been developed, and the clinical phenotypes associated with the histopathologic patterns of lymphoplasmacytic sclerosing pancreatitis and idiopathic duct-centric pancreatitis should be referred to as type 1 and type 2 AIP, respectively. Most importantly, in type 1 AIP, the pancreatic manifestations are associated with other extrapancreatic disorders, resembling an immunoglobulin G4 (IgG4)-related disease...
February 15, 2014: World Journal of Gastrointestinal Pathophysiology
Kimi Sumimoto, Kazushige Uchida, Takeo Kusuda, Toshiyuki Mitsuyama, Yutaku Sakaguchi, Toshiro Fukui, Mitsunobu Matsushita, Makoto Takaoka, Akiyoshi Nishio, Kazuichi Okazaki
BACKGROUND: Patients with type 1 autoimmune pancreatitis (AIP) have several immunologic and histologic abnormalities. It is known that depletion of B cells by rituximab is effective for treatment of IgG4-related disease (IgG4-RD) such as type 1 AIP, suggesting that B cells may be a key player in IgG4-RD. However, the role of regulatory B cells (Bregs) in type 1 AIP is unclear, and the objective of this paper is to clarify the role of Bregs in the pathophysiology of type 1 AIP by analyzing circulating Bregs...
May 2014: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
K Okazaki
Recently, autoimmune pancreatitis, a pancreatic manifestation of IgG4-related disease (IgG4-RD) has been recognized as a novel clinical entity associated with massive infiltration of IgG4-positive cells. The first international symposium on IgG4-RD endorsed the comprehensive nomenclature as IgG4-RD, which the Japanese research committee supported by the Ministry of Health, Labor and Welfare of Japan proposed in 2009, and proposed the individual nomenclatures for each organ system manifestations and the international pathologic consensus in 2011...
April 2014: Minerva Medica
Kazuichi Okazaki, Takashi Tomiyama, Toshiyuki Mitsuyama, Kimi Sumimoto, Kazushige Uchida
Recent studies suggested the existence of two subtypes of autoimmune pancreatitis (AIP): type 1 related with IgG4 as the pancreatic manifestation of IgG4-related disease (IgG4-RD), and type 2 related with a granulocytic epithelial lesion. Apart from type 2 AIP, the characteristic features of type 1 AIP are increased serum IgG4 levels, lymphoplasmacytic sclerosing pancreatitis (abundant infiltration of IgG4+ plasmacytes and lymphocytes, storiform fibrosis, and obliterative phlebitis), extra-pancreatic manifestations of IgG4-RD (e...
April 2014: Autoimmunity Reviews
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