title: Hepatocellular and extrahepatic cancer in patients with autoimmune hepatitis--a long-term follow-up study in 634 Swedish patients.
abstract: OBJECTIVE
  Cirrhosis is a well-known risk factor for hepatocellular cancer, but the true risk in autoimmune hepatitis (AIH) is scarcely studied. Other cancers may arise after prolonged use of immune-modulating drugs. The aim of this study was to investigate the cancer risk in a large cohort of AIH patients.
  
  
  METHODS
  Six hundred and thirty-four Swedish patients in a well-defined cohort were matched to the Cause of Death Registry and the Cancer Registry. Standard incidence ratios were calculated by relating the incidences in the cohort to an age-matched material from the Swedish background population.
  
  
  RESULTS
  A higher overall incidence of malignancies than the background population was found, counting from the date of diagnosis (standard incidence ratio (SIR) 2.08, 95% CI 1.68-2.55). The highest risk was found for hepatocellular carcinoma (HCC). We found 10 cases (4.0%) in 248 patients with cirrhosis, which gives an incidence rate of 0.3%. Standard incidence ratio for developing hepatobiliary cancer was 54.55 (95% CI 19.92-99.99). HCC only occurred in cirrhotic patients. There was also an increased risk for non-melanoma skin cancer (SIR 9.87, 95% CI 6.26-14.81).
  
  
  CONCLUSIONS
  A slightly enhanced risk for malignancies in general compared to the background population was found. The risk of hepatobiliary cancer was increased, but the annual risk over the observational period was well under the postulated 1.5% when surveillance in cirrhotic patients is considered to be cost-effective.
authors.affiliation: Departments of Medicine, Sections for Hepatology and Gastroenterology, Umeå University Hospital , Umeå , Sweden.
authors.forename: Åsa
authors.initials: Å
authors.lastname: Danielsson Borssén
authors.forename: Sven
authors.initials: S
authors.lastname: Almer
authors.forename: Hanne
authors.initials: H
authors.lastname: Prytz
authors.forename: Sven
authors.initials: S
authors.lastname: Wallerstedt
authors.forename: Inga-Lill
authors.initials: IL
authors.lastname: Friis-Liby
authors.forename: Annika
authors.initials: A
authors.lastname: Bergquist
authors.forename: Nils
authors.initials: N
authors.lastname: Nyhlin
authors.forename: Rolf
authors.initials: R
authors.lastname: Hultcrantz
authors.forename: Per
authors.initials: P
authors.lastname: Sangfelt
authors.forename: Ola
authors.initials: O
authors.lastname: Weiland
authors.forename: Stefan
authors.initials: S
authors.lastname: Lindgren
authors.forename: Hans
authors.initials: H
authors.lastname: Verbaan
authors.forename: Mårten
authors.initials: M
authors.lastname: Werner
mesh_terms: D000328:Adult; D000368:Aged; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology*; D015331:Cohort Studies; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000453:epidemiology*; D006801:Humans; D015994:Incidence; D008103:Liver Cirrhosis / Q000453:epidemiology*; D008113:Liver Neoplasms / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D012042:Registries; D012307:Risk Factors; D012878:Skin Neoplasms / Q000453:epidemiology*; D013548:Sweden / Q000453:epidemiology
keywords: autoimmune hepatitis; autoimmune liver disease; cancer; extrahepatic cancer; hepatocellular carcinoma