Diagnosis of Autoimmune Pancreatitis: The Evolution of Diagnostic Criteria for a Rare Disease

Source: Clinical Gastroenterology and Hepatology - Category: Gastroenterology Authors: Source Type: research

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Autoimmune pancreatitis (AIP) is a relatively newly discovered and rare disease that can be challenging to recognize and diagnose.1 Although a case report first described it in 1961, not until 1995 did it begin to be recognized as a clinical entity in a case series by Yoshida et  al.2 Type 1 AIP is the most common worldwide and is associated with IgG4-positive cells and histologic features described as lymphocytoplasmic sclerosing pancreatitis (LPSP) without granulocyte epithelial lesions, which are seen in type 2 AIP.
Source: Gastrointestinal Endoscopy - Category: Gastroenterology Authors: Tags: Original article Source Type: research
Differentiation between pancreatic cancer (PC) and focal form of autoimmune pancreatitis (AIP) is very challenging, with similar clinical presentations, laboratory results and morphologic imagings of US, CT, E...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Tags: Research Source Type: research
Authors: Mari A, Kadah A, Mahamid M, Sbeit W, Khoury T Abstract BACKGROUND: Autoimmune pancreatitis (AIP) is a rare disease that has been classified into two subtypes. Type 1 is believed to be mediated by immunoglobulin G4 (IgG4) and type 2 is related to granulocytic epithelial lesions, but the pathogenetic mechanisms in both are still unknown. The patho-mechanism of AIP type 1 is suggested to be secondary to autoimmunity or allergy due to the increased serum IgG4 and immunoglobulin E levels, abundant infiltration of IgG4, plasmacytes and lymphocytes in the pancreas, and fibrosis. Both types of AIP respond to stero...
Source: The Israel Medical Association Journal - Category: General Medicine Tags: Isr Med Assoc J Source Type: research
Xiaoli Fan1†, Dandan Yin2†, Ruoting Men1, Heng Xu2* and Li Yang1* 1Department of Gastroenterology and Hepatology, West China Hospital, Sichuan University, Chengdu, China 2Department of Laboratory Medicine, National Key Laboratory of Biotherapy/Collaborative Innovation Center for Biotherapy and Precision Medicine Key Laboratory of Sichuan Province, West China Hospital, Sichuan University, Chengdu, China The aim of this study was to investigate the influence of NUDT15 R139C and thiopurine S-methyltransferase (TPMT) on azathioprine (AZA) induced leukopenia in patients with autoimmune hepatitis (AIH) a...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
Authors: Vatier C, Vantyghem MC, Storey C, Jéru I, Christin-Maitre S, Fève B, Lascols O, Beltrand J, Carel JC, Vigouroux C, Bismuth E Abstract Lipodystrophic syndromes are rare diseases of genetic or acquired origin characterized by partial or generalized lack of body fat. Early detection and diagnosis are crucial to prevent and manage associated metabolic dysfunctions, i.e. insulin resistance, dyslipidemia, fatty liver and diabetes, and to provide appropriate genetic counselling. By means of several representative case studies, this article illustrates the diagnostic and management challenges of lipo...
Source: Current Medical Research and Opinion - Category: Research Tags: Curr Med Res Opin Source Type: research
We described a case of severe hypoproteinemia as a harbinger of Ménétrier's disease associated with AIP. The patient was successfully treated with octreotide and high-protein diet, which led to symptomatic remission and significant improvemen t in serum levels of albumin and recovery of the nutritional status. Thus, in AIP patients presenting with severe and persistent hypoproteinemia without apparent cause, clinicians need to consider Ménétrier's disease in the differential diagnosis. In this setting, endoscopic evaluation with histol ogical examination of gastric biopsy material, including a f...
Source: Revista da Associacao Medica Brasileira - Category: Journals (General) Source Type: research
AbstractAutoimmune pancreatitis (AIP) is a chronic fibroinflammatory disease of the pancreas that belongs to the spectrum of immunoglobulin G-subclass4-related diseases (IgG4-RD) and typically presents with obstructive jaundice. Idiopathic duct-centric pancreatitis (IDCP) is a closely related but distinct disease that mimics AIP radiologically but manifests clinically most commonly as recurrent acute pancreatitis in young individuals with concurrent inflammatory bowel disease. IgG4 levels are often elevated in AIP and normal in IDCP. Histologically, lymphoplasmacytic acinar inflammation and storiform fibrosis are seen in b...
Source: Digestive Diseases and Sciences - Category: Gastroenterology Source Type: research
We report nine new IMF cases recently seen at our Fibro-Inflammatory Disease Clinic and reviewed the IMF cases reported in the English language literature throughout 2006–2016. The purposes of our literature search were to assess the frequency of the association between IMF and other immune-medi ated disorders and to analyse which disorders most often coexist with IMF. Of our nine IMF cases, one was associated with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, one with large-vessel arteritis, three with idiopathic retroperitoneal fibrosis (one of which was IgG4-related) , one with pancreatitis an...
Source: Clinical Reviews in Allergy and Immunology - Category: Allergy & Immunology Source Type: research
Type 1 autoimmune pancreatitis (AIP) is clinically characterized by a response to steroid therapy. Despite having a favorable prognosis, AIP has a high relapse rate and factors predicting relapse in AIP patien...
Source: Orphanet Journal of Rare Diseases - Category: Internal Medicine Authors: Source Type: research
Abstract 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 t...
Source: Der Radiologe - Category: Radiology Authors: Tags: Radiologe Source Type: research
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