TY - JOUR
T1 - Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands
AU - van Gerven, Nicole M. F.
AU - Verwer, Bart J.
AU - Witte, Birgit I.
AU - van Erpecum, Karel J.
AU - van Buuren, Henk R.
AU - Maijers, Ingrid
AU - Visscher, Arjan P.
AU - Verschuren, Edwin C.
AU - van Hoek, Bart
AU - Coenraad, Minneke J.
AU - Beuers, Ulrich H. W.
AU - de Man, Robert A.
AU - Drenth, Joost P. H.
AU - den Ouden, Jannie W.
AU - Verdonk, Robert C.
AU - Koek, Ger H.
AU - Brouwer, Johannes T.
AU - Guichelaar, Maureen M. J.
AU - Vrolijk, Jan Maarten
AU - Mulder, Chris J. J.
AU - van Nieuwkerk, Carin M. J.
AU - Bouma, Gerd
PY - 2014
Y1 - 2014
N2 - 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
AB - 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
U2 - https://doi.org/10.3109/00365521.2014.946083
DO - https://doi.org/10.3109/00365521.2014.946083
M3 - Article
C2 - 25123213
SN - 0036-5521
VL - 49
SP - 1245
EP - 1254
JO - Scandinavian journal of gastroenterology
JF - Scandinavian journal of gastroenterology
IS - 10
ER -