scholarly journals Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

2017 ◽  
Vol 2017 ◽  
pp. 1-9 ◽  
Author(s):  
Matthias Buechter ◽  
Paul Manka ◽  
Falko Markus Heinemann ◽  
Monika Lindemann ◽  
Benjamin Juntermanns ◽  
...  

Introduction. Most investigations on autoimmune pancreatitis (AIP) were published on Asian cohorts while those on Caucasians are limited. However, there might be differences related to the origin.Patients and Methods. We analyzed 36 patients and compared type 1 (AIP1) with type 2 (AIP2).Results. The majority of patients suffered from AIP1 (55.6%). AIP1 patients were significantly older than AIP2 patients (54.4 versus 40.8 years). Moreover, 85.0% of AIP1 patients had concurrent autoimmune cholangitis (AIC) while 18.8% of AIP2 patients suffered from overlap to ulcerative colitis (UC). However, AIP1 patients revealed a cholestatic course and had significantly higher immunoglobulin G4 levels (IgG4). When compared to allele frequencies in healthy controls, in patients with AIP1 HLA-B8 reached statistical significance. Response to steroids was excellent in both groups, but we noticed high rates of relapse especially in AIP1 patients. Finally, 3 patients with AIP1 were diagnosed with cholangiocellular carcinoma (CCC).Conclusion. In contrast to Asian studies, we found an almost equal distribution of AIP1 and AIP2 patients in our German cohort. AIP2 patients were younger and mostly of female gender whereas AIP1 patients revealed higher IgG4 levels and involvement of the biliary tract in sense of IgG4-associated cholangitis.

2020 ◽  
Vol 95 (2) ◽  
pp. 114-118
Author(s):  
So Hyun Kim ◽  
Seung-Ho Baek ◽  
Hye Yeong Kim ◽  
Su Jin Choi ◽  
Ji Hoon Kim ◽  
...  

There are two forms of autoimmune pancreatitis (AIP). Type 1 is associated with immunoglobulin G4 (IgG4)-related systemic fibro- inflammatory disease, whereas type 2 AIP is localized to the pancreas and not associated with IgG4. The number of children presenting with type 2 AIP has recently increased. Here, we report a case of type 2 AIP in a 16-year-old adolescent who presented with clinical acute pancreatitis and associated pancreatic masses. He was diagnosed with type 2 AIP based on pancreatic biopsy results showing granulocytic epithelial lesions and supportive radiological imaging and steroid responsiveness.


Author(s):  
Kauê de Melo Souza ◽  
Lucas Facco ◽  
Amanda Alves Fecury ◽  
Maria Helena Mendonça de Araújo ◽  
Euzébio de Oliveira ◽  
...  

Diabetes mellitus is a succession of different types of disorders in metabolism that are characterized by causing a high rate of blood sugar. Because it is a disease with genetic factors type 1 diabetes has as main risk factor heredity, while type 2 diabetes besides these factors, includes obesity, high blood pressure, poor food education and advancing age. This study aims to show the number of cases of type 1 and 2 diabetes diagnosed in Amapá with the variables gender, age group, sedentary lifestyle, overweight, smoking, between 2007 and 2012. The data for the research were taken from the computer department of SUS, DATASUS (http://datasus.saude.gov.br). Type 1 and 2 diabetes mellitus (DM1 and DM2) are diseases that are tied to disturbances in production or in the efficient use of insulin. Smoking, as well as sedentary lifestyle and overweight are important risk factors for the development of DM2. Type 2 diabetes mellitus provides the development of various organic nerve lesions. In addition, DM2, through its chronicity, enables the development of retinopathies, nephropathies and other conditions negative to the individual’s health.


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 ◽  
...  

2017 ◽  
Vol 11 (2) ◽  
pp. 329-334
Author(s):  
Nathan T. Kolasinski ◽  
Marc T. Johannsen ◽  
Justin R. Hollon

Type 2 autoimmune pancreatitis, an increasingly recognized etiology of pancreatitis in patients less than 20 years old, has characteristically been diagnosed with the histological finding of duct-centric pancreatitis in a patient who lacks elevated serum immunoglobulin G4. We present the case of a nonobese 15-year-old male, without any chronic medical conditions, who presented with the chief complaint of abdominal pain. The laboratory study results were remarkable for a lipase level of 5,419 U/L and a γ-glutamyl transferase level of 373 U/L. Magnetic resonance cholangiopancreatography revealed delayed contrast enhancement of the pancreas, diffuse parenchymal enlargement, and lack of normal lobulation. The patient’s serum immunoglobulin G4 level was found to be 66 mg/dL, which was within normal limits and supportive of a diagnosis of type 2 autoimmune pancreatitis. Despite the absence of intestinal complaints, the patient underwent subsequent endoscopy due to the correlation of type 2 autoimmune pancreatitis with inflammatory bowel disease that has been described in recent literature. Pan-colonic mild colitis was visualized, and the patient began treatment with steroids, to which he quickly responded. Performing endoscopy on this patient allowed for confident initiation of early therapy for both autoimmune pancreatitis and inflammatory bowel disease, and may have limited further surgical intervention and disease progression. For these reasons, this case highlights the utility of endoscopy in pediatric patients with suspected type 2 autoimmune pancreatitis, even in the absence of intestinal symptoms.


2012 ◽  
Vol 19 (6) ◽  
pp. 793-803 ◽  
Author(s):  
Prue J Hardefeldt ◽  
Senarath Edirimanne ◽  
Guy D Eslick

The aim of this meta-analysis was to collate and analyse all primary observational studies investigating the risk of breast cancer (BC) associated with diabetes. In addition, we aimed to complete subgroup analyses by both type of diabetes and gender of study participants to further clarify the origin of any such association between the two. Studies were obtained from a database search of MEDLINE, EMBASE, PubMed, Current Contents Connect and Google Scholar with additional cross-checking of reference lists. Collated data were assessed for heterogeneity and a pooled odds ratio (OR) calculated. Forty-three studies were included in the meta-analysis with 40 studies investigating BC in women and six studies investigating BC in men. Overall, we found a significantly increased risk of BC associated with diabetes in women (OR 1.20, 95% confidence interval (CI) 1.13–1.29). After subgroup analysis by type of diabetes, the association was unchanged with type 2 diabetes (OR 1.22, 95% CI 1.07–1.40) and nullified with gestational diabetes (OR 1.06, 95% CI 0.79–1.40). There were insufficient studies to calculate a pooled OR of the risk of BC associated with type 1 diabetes. There was an increased risk of BC in males with diabetes mellitus; however, the results did not reach statistical significance (OR 1.29, 95% CI 0.99–1.67). In conclusion, diabetes increases the risk of BC in women. This association is confirmed in women with type 2 diabetes and supports the hypothesis that diabetes is an independent risk factor for BC.


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