title: Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands.
abstract: OBJECTIVE
  Epidemiological data on autoimmune hepatitis (AIH) are scarce. In this study, we determined the clinical and epidemiological characteristics of AIH patients in the Netherlands (16.7 million inhabitants).
  
  
  METHODS
  Clinical characteristics were collected from 1313 AIH patients (78% females) from 31 centers, including all eight academic centers in the Netherlands. Additional data on ethnicity, family history and symptoms were obtained by the use of a questionnaire.
  
  
  RESULTS
  The prevalence of AIH was 18.3 (95% confidential interval [CI]: 17.3-19.4) per 100,000 with an annual incidence of 1.1 (95% CI: 0.5-2) in adults. An incidence peak was found in middle-aged women. At diagnosis, 56% of patients had fibrosis and 12% cirrhosis in liver biopsy. Overall, 1% of patients developed HCC and 3% of patients underwent liver transplantation. Overlap with primary biliary cirrhosis and primary sclerosing cholangitis was found in 9% and 6%, respectively. The clinical course did not differ between Caucasian and non-Caucasian patients. Other autoimmune diseases were found in 26% of patients. Half of the patients reported persistent AIH-related symptoms despite treatment with a median treatment period of 8 years (range 1-44 years). Familial occurrence was reported in three cases.
  
  
  CONCLUSIONS
  This is the largest epidemiological study of AIH in a geographically defined region and demonstrates that the prevalence of AIH in the Netherlands is uncommon. Although familial occurrence of AIH is extremely rare, our twin data may point towards a genetic predisposition. The high percentage of patients with cirrhosis or fibrosis at diagnosis urges the need of more awareness for AIH.
authors.affiliation: Department of Gastroenterology and Hepatology, VU University Medical Center , Amsterdam , The Netherlands.
authors.forename: Nicole M F
authors.initials: NM
authors.lastname: van Gerven
authors.forename: Bart J
authors.initials: BJ
authors.lastname: Verwer
authors.forename: Birgit I
authors.initials: BI
authors.lastname: Witte
authors.forename: Karel J
authors.initials: KJ
authors.lastname: van Erpecum
authors.forename: Henk R
authors.initials: HR
authors.lastname: van Buuren
authors.forename: Ingrid
authors.initials: I
authors.lastname: Maijers
authors.forename: Arjan P
authors.initials: AP
authors.lastname: Visscher
authors.forename: Edwin C
authors.initials: EC
authors.lastname: Verschuren
authors.forename: Bart
authors.initials: B
authors.lastname: van Hoek
authors.forename: Minneke J
authors.initials: MJ
authors.lastname: Coenraad
authors.forename: Ulrich H W
authors.initials: UH
authors.lastname: Beuers
authors.forename: Robert A
authors.initials: RA
authors.lastname: de Man
authors.forename: Joost P H
authors.initials: JP
authors.lastname: Drenth
authors.forename: Jannie W
authors.initials: JW
authors.lastname: den Ouden
authors.forename: Robert C
authors.initials: RC
authors.lastname: Verdonk
authors.forename: Ger H
authors.initials: GH
authors.lastname: Koek
authors.forename: Johannes T
authors.initials: JT
authors.lastname: Brouwer
authors.forename: Maureen M J
authors.initials: MM
authors.lastname: Guichelaar
authors.forename: Jan Maarten
authors.initials: JM
authors.lastname: Vrolijk
authors.forename: Chris J J
authors.initials: CJ
authors.lastname: Mulder
authors.forename: Carin M J
authors.initials: CM
authors.lastname: van Nieuwkerk
authors.forename: Gerd
authors.initials: G
authors.lastname: Bouma
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000410:Alanine Transaminase / Q000097:blood; D000469:Alkaline Phosphatase / Q000097:blood; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000097:blood; D044466:Asian People / Q000706:statistics & numerical data; D044383:Black People / Q000706:statistics & numerical data; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology*; D002648:Child; D002675:Child, Preschool; D005221:Fatigue / Q000209:etiology; D005260:Female; D019693:Hepatitis, Autoimmune / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology* / Q000235:genetics; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D015994:Incidence; D007565:Jaundice / Q000209:etiology; D008103:Liver Cirrhosis / Q000453:epidemiology; D008113:Liver Neoplasms / Q000453:epidemiology*; D008297:Male; D008875:Middle Aged; D009426:Netherlands / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies; D012737:Sex Factors; D013020:South America / Q000208:ethnology; D011795:Surveys and Questionnaires; D044465:White People / Q000706:statistics & numerical data; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D000410:Alanine Transaminase; D000469:Alkaline Phosphatase
keywords: autoimmune hepatitis; cirrhosis; diagnosis; diagnostic scoring systems; epidemiology; hepatocellular carcinoma; prevalence; transplantation