title: Diagnosis, treatment and long-term outcomes of autoimmune pancreatitis in Spain based on the International Consensus Diagnostic Criteria: A multi-centre study.
abstract: OBJECTIVE
  Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that has been reported worldwide for the last two decades. The aim of this study is to analyse the clinical profile of patients from Spain with AIP, as well as treatments, relapses and long-term outcomes.
  
  
  METHODS
  Data from 59 patients with suspected AIP that had been diagnosed in 15 institutions are retrospectively analysed. Subjects are classified according to the International Consensus Diagnostic Criteria (ICDC). Patients with type 1 AIP (AIP1) and type 2 AIP (AIP2) are compared. Kaplan-Meier methodology is used to estimate the overall survival without relapses.
  
  
  RESULTS
  Fifty-two patients met ICDC, 45 patients were AIP1 (86.5%). Common manifestations included abdominal pain (65.4%) and obstructive jaundice (51.9%). Diffuse enlargement of pancreas was present in 51.0%; other organ involvement was present in 61.5%. Serum IgG4 increased in 76.7% of AIP1 patients vs. 20.0% in AIP2 (p = 0.028). Tissue specimens were obtained in 76.9%. Initial successful treatment with steroids or surgery was achieved in 79.8% and 17.3%, respectively. Maintenance treatment was given in 59.6%. Relapses were present in 40.4% of AIP1, with a median of 483 days. Successful long-term remission was achieved in 86.4%.
  
  
  CONCLUSIONS
  AIP1 is the most frequent form of AIP in Spain in our dataset. Regularly, ICDC allows AIP diagnosis without the need for surgery. Steroid and chirurgic treatments were effective and safe in most patients with AIP, although maintenance was required many times because of their tendency to relapse. Long-term serious consequences were uncommon.
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain. Electronic address: lopez_antser@gva.es.
authors.forename: Antonio
authors.initials: A
authors.lastname: López-Serrano
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Crespo
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Isabel
authors.initials: I
authors.lastname: Pascual
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Silvia
authors.initials: S
authors.lastname: Salord
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Federico
authors.initials: F
authors.lastname: Bolado
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Andrés J
authors.initials: AJ
authors.lastname: Del-Pozo-García
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Lucas
authors.initials: L
authors.lastname: Ilzarbe
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Enrique
authors.initials: E
authors.lastname: de-Madaria
authors.affiliation: Department of Gastroenterology, Hospital Universitari Dr. Peset, University of Valencia, Valencia, Spain.
authors.forename: Eduardo
authors.initials: E
authors.lastname: Moreno-Osset
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000628:therapy; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005500:Follow-Up Studies; D053208:Kaplan-Meier Estimate; D050500:Pancreatitis, Chronic / Q000175:diagnosis / Q000628:therapy; D012189:Retrospective Studies; D013030:Spain; D016896:Treatment Outcome; D003245:Consensus Statements as Topic
keywords: Autoimmune diseases; Chronic; Diagnosis; Immunoglobulin G; Pancreatitis; Therapeutics