title: Intraductal papillary mucinous neoplasm of the pancreas, one manifestation of a more systemic disease?
abstract: BACKGROUND
  Several studies have demonstrated a high prevalence of extrapancreatic malignancies, and an association with autoimmune pancreatitis in patients with intraductal papillary mucinous neoplasm (IPMN). We hypothesized that IPMNs were associated with an increase rate of systemic diseases.
  
  
  METHODS
  From 1996 to 2013, a retrospective analysis of a prospectively collected database was performed and supplemented with electronic medical charts review.
  
  
  RESULTS
  Two hundred twenty extrapancreatic malignancies were found in 185 patients (22%) compared with expected 5% in the general population. Colorectal, lung, and renal cell carcinoma had significant observed/expected ratios (P < .0001). One hundred ten synchronous autoimmune diseases were found in 96 patients (11%). Systemic lupus erythematosus, rheumatoid arthritis, and inflammatory bowel disease showed statistically significant observed/expected ratios (P < .0001, .01, and <.0001, respectively). There was no impact of immunosuppressive treatment on the IPMN subtype and malignancy rate.
  
  
  CONCLUSIONS
  IPMN are associated with surprisingly high rates of autoimmune diseases suggesting that IPMN might be 1 manifestation of a more systemic disease.
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Alexandra M
authors.initials: AM
authors.lastname: Roch
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Carlo Maria
authors.initials: CM
authors.lastname: Rosati
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Jessica L
authors.initials: JL
authors.lastname: Cioffi
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Eugene P
authors.initials: EP
authors.lastname: Ceppa
authors.affiliation: Division of Gastroenterology, Department of Medicine, Indiana University Hospital, Indianapolis, IN, USA.
authors.forename: John M
authors.initials: JM
authors.lastname: DeWitt
authors.affiliation: Digestive Disease Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, United Arab Emirates.
authors.forename: Mohammad A
authors.initials: MA
authors.lastname: Al-Haddad
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Michael G
authors.initials: MG
authors.lastname: House
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Nicholas J
authors.initials: NJ
authors.lastname: Zyromski
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA.
authors.forename: Attila
authors.initials: A
authors.lastname: Nakeeb
authors.affiliation: Department of Surgery, Indiana University School of Medicine, 980 West Walnut Street C522, Indianapolis, IN, 46202, USA. Electronic address: maxschmi@iupui.edu.
authors.forename: C Max
authors.initials: CM
authors.lastname: Schmidt
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001327:Autoimmune Diseases / Q000453:epidemiology* / Q000473:pathology*; D021441:Carcinoma, Pancreatic Ductal / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D002291:Carcinoma, Papillary / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D015994:Incidence; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology* / Q000473:pathology*; D012189:Retrospective Studies
keywords: Autoimmune diseases; Extrapancreatic malignancies; Incidence; Intraductal papillary mucinous neoplasm