title: Mortality and extraintestinal cancers in patients with primary sclerosing cholangitis and inflammatory bowel disease.
abstract: BACKGROUND
  Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) frequently co-occur. PSC is associated with increased risk for colorectal cancer (CRC). However, whether PSC is associated with increased risk of extraintestinal cancers or affects mortality in an IBD cohort has not been examined previously.
  
  
  METHODS
  In a multi-institutional IBD cohort of IBD, we established a diagnosis of PSC using a novel algorithm incorporating narrative and codified data with high positive and negative predictive value. Our primary outcome was occurrence of extraintestinal and digestive tract cancers. Mortality was determined through monthly linkage to the social security master death index.
  
  
  RESULTS
  In our cohort of 5506 patients with CD and 5522 patients with UC, a diagnosis of PSC was established in 224 patients (2%). Patients with IBD-PSC were younger and more likely to be male compared to IBD patients without PSC; three-quarters had UC. IBD-PSC patients had significantly increased overall risk of cancers compared to patients without PSC (OR 4.36, 95% CI 2.99-6.37). Analysis of specific cancer types revealed that a statistically significant excess risk for digestive tract cancer (OR 10.40, 95% CI 6.86-15.76), pancreatic cancer (OR 11.22, 95% CI 4.11-30.62), colorectal cancer (OR 5.00, 95% CI 2.80-8.95), and cholangiocarcinoma (OR 55.31, 95% CI 22.20-137.80) but not for other solid organ or hematologic malignancies.
  
  
  CONCLUSIONS
  PSC is associated with increased risk of colorectal and pancreatobiliary cancer but not with excess risk of other solid organ cancers.
authors.affiliation: Division of Gastroenterology, Massachusetts General Hospital, Boston, MA, United States; Harvard Medical School, Boston, MA, United States. Electronic address: aananthakrishnan@partners.org.
authors.forename: Ashwin N
authors.initials: AN
authors.lastname: Ananthakrishnan
authors.affiliation: Research IS and Computing, Partners HealthCare, Charlestown, MA, United States.
authors.forename: Andrew
authors.initials: A
authors.lastname: Cagan
authors.affiliation: Research IS and Computing, Partners HealthCare, Charlestown, MA, United States.
authors.forename: Vivian S
authors.initials: VS
authors.lastname: Gainer
authors.affiliation: Department of Biostatistics, Harvard School of Public Health, Boston, MA, United States.
authors.forename: Su-Chun
authors.initials: SC
authors.lastname: Cheng
authors.affiliation: Department of Biostatistics, Harvard School of Public Health, Boston, MA, United States.
authors.forename: Tianxi
authors.initials: T
authors.lastname: Cai
authors.affiliation: Massachusetts Institute of Technology, Cambridge, MA, United States.
authors.forename: Peter
authors.initials: P
authors.lastname: Szolovits
authors.affiliation: Harvard Medical School, Boston, MA, United States; Center for Systems Biology, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Stanley Y
authors.initials: SY
authors.lastname: Shaw
authors.affiliation: i2b2 National Center for Biomedical Computing, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Susanne
authors.initials: S
authors.lastname: Churchill
authors.affiliation: Harvard Medical School, Boston, MA, United States; Division of Rheumatology, Allergy and Immunology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Elizabeth W
authors.initials: EW
authors.lastname: Karlson
authors.affiliation: Harvard Medical School, Boston, MA, United States; Research IS and Computing, Partners HealthCare, Charlestown, MA, United States; Department of Neurology, Massachusetts General Hospital, Boston, MA, United States.
authors.forename: Shawn N
authors.initials: SN
authors.lastname: Murphy
authors.affiliation: Harvard Medical School, Boston, MA, United States; Children's Hospital Boston, Boston, MA, United States; i2b2 National Center for Biomedical Computing, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Isaac
authors.initials: I
authors.lastname: Kohane
authors.affiliation: Harvard Medical School, Boston, MA, United States; Division of Rheumatology, Allergy and Immunology, Brigham and Women's Hospital, Boston, MA, United States.
authors.forename: Katherine P
authors.initials: KP
authors.lastname: Liao
mesh_terms: D000328:Adult; D002760:Cholangiopancreatography, Endoscopic Retrograde; D015209:Cholangitis, Sclerosing / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D018450:Disease Progression; D016099:Endoscopy, Gastrointestinal; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D015212:Inflammatory Bowel Diseases / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D008297:Male; D008404:Massachusetts / Q000453:epidemiology; D008875:Middle Aged; D009369:Neoplasms / Q000209:etiology / Q000401:mortality*; D011379:Prognosis; D018570:Risk Assessment; D012307:Risk Factors; D015996:Survival Rate / Q000639:trends
keywords: Cancer;; Colorectal cancer; Crohn's disease;; Sclerosing cholangitis;; Ulcerative colitis;