title: Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience.
abstract: 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.
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Buechter
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany; Division of Transplantation Immunology and Mucosal Biology, King's College London, London, UK.
authors.forename: Paul
authors.initials: P
authors.lastname: Manka
authors.affiliation: Institute of Transfusion Medicine, University Hospital Essen, Essen, Germany.
authors.forename: Falko Markus
authors.initials: FM
authors.lastname: Heinemann
authors.affiliation: Institute of Transfusion Medicine, University Hospital Essen, Essen, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Lindemann
authors.affiliation: Department of General, Visceral, and Transplantation Surgery, University Hospital Essen, Essen, Germany.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Juntermanns
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.
authors.forename: Ali
authors.initials: A
authors.lastname: Canbay
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.
authors.forename: Guido
authors.initials: G
authors.lastname: Gerken
authors.affiliation: Department of Gastroenterology and Hepatology, University Hospital Essen, Essen, Germany.
authors.forename: Alisan
authors.identifier: 0000-0002-2823-6774
authors.initials: A
authors.lastname: Kahraman