title: [Autoimmune pancreatitis--treatment and pitfalls in diagnostics].
abstract: 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.
  
  
  OBJECTIVE
  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.
  
  
  RESULTS
  Key characteristics of AIP are recurrent jaundice due to obstructed bile ducts, histological evidence of fibrosis, a lymphoplasmocytic or granulocytic infiltrate and the response to steroid therapy. There are two distinctive forms of AIP: type I or lymphoplasmocytic sclerosing pancreatitis and type II or idiopathic duct centric pancreatitis. The IgG4 positive AIP type I belongs to the group of IgG4-related systemic diseases. Diagnosis of AIP is established according to the international consensus diagnostic criteria (ICDC) or HISORt (mnemonic standing for histology, imaging, serology, other organ involvement and response to therapy) criteria. Differentiation from pancreatic adenocarcinoma can be challenging. The standard treatment consists of corticosteroids and in some cases azathioprine can be added. In refractory disease rituximab is a further option. Treatment is indicated in patients with jaundice, systemic manifestation or persistent pain.
  
  
  CONCLUSIONS
  Although AIP is increasingly being identified, the differentiation from pancreatic adenocarcinoma still remains difficult and in cases of a suspicion of neoplasia, resection should be favored. It can successfully be treated conservatively with steroids and rituximab.
authors.affiliation: Ambulanz für Pankreaserkrankungen, II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675, München, Deutschland, sebastian.rasch@lrz.tum.de.
authors.forename: S
authors.initials: S
authors.lastname: Rasch
authors.forename: V
authors.initials: V
authors.lastname: Phillip
authors.forename: G
authors.initials: G
authors.lastname: Weirich
authors.forename: I
authors.initials: I
authors.lastname: Esposito
authors.forename: J
authors.initials: J
authors.lastname: Gaa
authors.forename: R M
authors.initials: RM
authors.lastname: Schmid
authors.forename: H
authors.initials: H
authors.lastname: Algül
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use*; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy*; D001379:Azathioprine / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D005189:False Positive Reactions; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010195:Pancreatitis / Q000175:diagnosis* / Q000188:drug therapy*; D000069283:Rituximab
chemical_list: D000305:Adrenal Cortex Hormones; D000893:Anti-Inflammatory Agents; D058846:Antibodies, Monoclonal, Murine-Derived; D007166:Immunosuppressive Agents; D000069283:Rituximab; D001379:Azathioprine
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