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Abstract
Background and aims. 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. Conclusion. 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.
Acknowledgment
Collaborators: L.C. Baak (Onze Lieve Vrouwe Gasthuis, Amsterdam), M. Klemt-Kropp (Medisch Centrum Alkmaar, Alkmaar), J.J.M. van Meyel (Sint Lucas Andreas Ziekenhuis, Amsterdam), R.K. Linskens (St. Anna ziekenhuis, Geldrop), J.C. Kneppelhout (St. Anna ziekenhuis, Geldrop), J.Ph. Kuyvenhoven (Kennemer Gasthuis, Haarlem), E.J.M. van Geenen (Bronovo, den Haag,), M.J. Wagtmans (Rode Kruis ziekenhuis, Beverwijk), D.L. Cahen (ziekenhuis Amstelland, Amstelveen) F.H.J. Wolfhagen (Tweesteden ziekenhuis, Tilburg), P.J. Kingma (Tergooiziekenhuizen, Hilversum), J.M.L. de Vree (Medisch Centrum Leeuwarden, Leeuwarden), R.J.L.F. Loffeld (Zaans Medisch Centrum, Zaandam), J.M. Vrolijk (Rijnstate ziekenhuis, Arnhem), P.W. Friederich (Meander Medisch Centrum, Amersfoort), T.C.M.A. Schreuder (Slingeland ziekenhuis, Doetichem), A.W.M. van Milligen de Wit (Amphia ziekenhuis, Breda), M.A. Alleman (Isala, Zwolle), A. Bhalla (Hagaziekenhuis, Den haag), P.H.G.M. Stadhouders (St Antonius ziekenhuis, Nieuwegein), M.A.M.T. Verhagen (Diakonessenhuis, Utrecht).
Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.