Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands

作者：Nicole M. F. van Gerven, Bart J. Verwer, Birgit Ilja Lissenberg-Witte, Karel J. van Erpecum, Henk R. van Buuren, Ingrid Maijers, Arjan P. Visscher, Edwin C. Verschuren, Bart van Hoek, Minneke J. Coenraad, Ulrich H. Beuers, Robert Auke de Man, Joost P.H. Drenth, Jannie W. den Ouden, Robert C. Verdonk, Ger H. Koek, Johannes T. Brouwer, Maureen M. J. Guichelaar, Jan Maarten Vrolijk, Chris J. Mulder, Carin M.J. van Nieuwkerk, Gerd Bouma, On behalf of the Dutch Autoimmune hepatitis STUDY group (see list of contributors at the end of the Manuscript) · 发表于：Scandinavian Journal of Gastroenterology · 年份：2014 · DOI：10.3109/00365521.2014.946083 · 被引用次数：190 · 研究领域：Liver Diseases and Immunity、IgG4-Related and Inflammatory Diseases、Pediatric Hepatobiliary Diseases and Treatments

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 occurren...