Tu1475 Immunogenetic Profile and IgG4 Levels Discriminate Among Autoimmune Pancreatitis Type 1 and Autoimmune Pancreatitis Type 2. A Multicentric Perspective Study in Caucasian Population

2016 ◽  
Vol 150 (4) ◽  
pp. S912
Author(s):  
Elisabetta Goni ◽  
Nicolo' De Pretis ◽  
Antonio Amodio ◽  
Francesco Bonatti ◽  
Davide Martorana ◽  
...  
Gut ◽  
2013 ◽  
Vol 62 (9) ◽  
pp. 1373-1380 ◽  
Author(s):  
Terumi Kamisawa ◽  
Suresh T Chari ◽  
Markus M Lerch ◽  
Myung-Hwan Kim ◽  
Thomas M Gress ◽  
...  

2018 ◽  
Author(s):  
Allison L Yang ◽  
Julia McNabb-Baltar

Autoimmune pancreatitis (AIP) is a subcategory of chronic pancreatitis that is highly responsive to steroids. The term was first proposed in 1995 by Yoshida and colleagues, and since its discovery, the diagnosis of AIP has dramatically increased. AIP is a chronic fibroinflammatory disease characterized by lymphoplasmacytic infiltrates and fibrosis on histology. There are two distinct subtypes: type 1 AIP is the pancreatic manifestation of a systemic serum immunoglobulin G subtype 4–related disease (IgG4-RD) and type 2 AIP is described clinically as idiopathic duct-centric pancreatitis and has no association with IgG4. Clinically, AIP presents most commonly as obstructive jaundice in type 1 AIP and can present as acute pancreatitis in type 2 AIP. The diagnostic criteria include histology, imaging findings, and responsiveness to steroids as well as laboratory findings and other organ involvement. The mainstay of treatment is steroid therapy, with immunomodulators such as rituximab used for maintenance or relapsing disease. Long-term complications of AIP include pancreatic insufficiency and are often associated with relapsing disease. This review contains 45 references, 1 figure, and 2 tables. Key Words: autoimmune pancreatitis, chronic pancreatitis, EUS-guided biopsy, IgG4, immunomodulatory, obstructive jaundice, pancreas mass, steroid


2021 ◽  
Author(s):  
Sara Nikolic ◽  
Poya Ghorbani ◽  
Raffaella Pozzi Mucelli ◽  
Sam Ghazi ◽  
Francisco Baldaque- Silva ◽  
...  

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.


2014 ◽  
Vol 50 (7) ◽  
pp. 805-815 ◽  
Author(s):  
Shigeyuki Kawa ◽  
◽  
Kazuichi Okazaki ◽  
Kenji Notohara ◽  
Mamoru Watanabe ◽  
...  

Pancreatology ◽  
2017 ◽  
Vol 17 (4) ◽  
pp. S40
Author(s):  
Tsukasa Ikeura ◽  
Kota Kato ◽  
Kazushige Uchida ◽  
Makoto Takaoka ◽  
Akiyoshi Nishio ◽  
...  

2018 ◽  
Vol 46 (4) ◽  
pp. 330-337
Author(s):  
S. V. Lishchuk ◽  
Е. A. Dubova ◽  
K. А. Pavlov ◽  
Yu. D. Udalov

Rationale: In the recent years, an increased interest to autoimmune pancreatitis (AIP) has been seen, related to growing diagnostic potential. In its turn, this leads to an increase in numbers of diagnosed AIP cases. At present, two types of AIP have been described with diverse clinical manifestation and morphology of the pancreas. However, the reproducibility of the differential diagnosis between AIP type 1 and 2 is low even among pancreatic pathologists.Aim: To identify criteria for the morphologic diagnosis of AIP type 1 and 2.Materials and methods: A morphological study of biopsy and surgical specimens from 26 patients with AIP was performed. There were 22 cases of AIP type 1 and 4 cases of AIP type 2. In addition to hematoxylin eosin staining of the specimens, immunohistochemistry was used with counting of CD138+ absolute numbers, determination of IgG+ and IgG4+ cells in the inflammatory infiltrates, as well as the ratios of IgG4+/IgG+ and IgG4+/CD138+ cells.Results: AIP type 1 was characterized by storiform fibrosis of the pancreatic tissue (81.8% cases), involving the parapancreatic fat tissue, by moderateto-severe lymphoplasmocytic infiltration and signs of obliterative/non-obliterative phlebitis. Type 2 AIP was characterized by severe fibrosis with predominantly periductal (centrilobular) fibrosis and mild chronic inflammatory infiltration of the pancreas, while there was no extension of fibrosis and inflammatory infiltration to the parapancreatic tissues in any case. The mean number of CD138+ cell in AIP type 1 was 101.2 ± 27.9 per 1 high-power field (HPF), and in AIP type 2, it was 42.8 ± 20.9 per 1 HPF. The mean absolute number of IgG+ cells in AIP type 1 was 99.6 ± 25.7 per 1 HPF, whereas in AIP type 2, 42.1 ± 20.8 per 1 HPF. In AIP type 1, the mean number of IgG4+ plasmatic cells in the infiltrates was 74.5 ± 27.2 per 1 HPF, whereas in AIP type 2, it was 3.4 ± 2.7 per 1 HPF. The IgG4+/IgG+ ratio was 75 ± 12.6% vs. 8.4 ± 6.2%, and the IgG4+/CD138+ ratio was 72.4 ± 12.3% vs. 8.3 ± 5.9% in AIP type 1 and type 2, respectively.Conclusion: For the differential diagnosis of type 1 and 2 AIP, it is necessary to take into consideration not only typical histological abnormalities, but also the numbers of CD138+, IgG+ and IgG4+ cells within the inflammatory infiltrate, as well as the IgG4+/IgG+ and IgG4+/CD138+ ratios.


2017 ◽  
Vol 5 (4) ◽  
pp. 232470961773424 ◽  
Author(s):  
Chidinma Onweni ◽  
Harika Balagoni ◽  
Jennifer M. Treece ◽  
Emmanuel Addo Yobo ◽  
Archi Patel ◽  
...  

A middle-aged man presents with acute pancreatitis of unknown etiology and is found to have a presentation consistent with the diagnosis of type 2 autoimmune pancreatitis (AIP). AIP is a group of rare heterogeneous diseases that are challenging to diagnose. There are 2 types of AIP. Type 1 disease is the more common worldwide than type 2 AIP. While type 1 AIP is associated with IgG4-positive antibodies, type 2 AIP is IgG4 antibody negative. Both types of AIP are responsive to corticosteroid treatment. Although type 1 AIP has more extrapancreatic manifestations and more commonly relapses, this is a case of a patient with type 2 AIP with inflammatory bowel disease and relapsing course.


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