title: Epidemiology, clinical presentation, diagnosis and treatment of autoimmune pancreatitis: A retrospective analysis of 53 patients.
abstract: BACKGROUND
  Most of the data about epidemiology, clinical presentation and treatment of autoimmune pancreatitis (AIP) is based on case series or small study groups. We therefore analyzed all cases of AIP treated at our clinic retrospectively.
  
  
  METHODS
  We searched our clinical database for the diagnosis pancreatitis between January 2007 and June 2014, selected patients with AIP and entered all relevant information in a database for statistical analysis.
  
  
  RESULTS
  In total 53 patients with AIP were treated at our institution, 62% with type 1 and 23% with type 2 AIP. Gender distribution was male/female 3.1:1 for type 1 and 1:1.2 for type 2 AIP. The median age was 63.0 and 32.5 years for type 1 and type 2 AIP, respectively. The most common symptom is abdominal pain particular in patients with type 2 AIP whereas jaundice was only apparent in patients with type 1 AIP. The international diagnostic criteria seem to facilitate diagnosis of AIP as unnecessary pancreatic surgery in patients with AIP decreases. In 62.6% of the patients therapy was indicated and 84.8% showed a response to initial therapy with steroids. Recurring disease occurred in 28.3% of the cases but only 3.8% suffered a second relapse. Permanent maintenance therapy with steroids or additional therapy with immunomodulatory drugs is successful in recurring disease.
  
  
  CONCLUSIONS
  Our data further corroborate previous findings on epidemiology, clinical presentation and treatment of AIP. AIP is a well manageable autoimmune disease in most patients. Better biopsy techniques and simplified diagnostic criteria might further alleviate diagnosis of AIP.
authors.affiliation: II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 München, Germany. Electronic address: sebastian.rasch@lrz.tum.de.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Rasch
authors.affiliation: II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 München, Germany.
authors.forename: Veit
authors.initials: V
authors.lastname: Phillip
authors.affiliation: II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 München, Germany.
authors.forename: Roland M
authors.initials: RM
authors.lastname: Schmid
authors.affiliation: II. Medizinische Klinik und Poliklinik, Klinikum rechts der Isar, Technische Universität München, Ismaninger Straße 22, 81675 München, Germany.
authors.forename: Hana
authors.initials: H
authors.lastname: Algül
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Autoimmune pancreatitis; Chronic pancreatitis; Idiopathic duct centric pancreatitis; IgG4; IgG4-related disease; Lymphoplasmacytic sclerosing pancreatitis