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Scand J Gastroenterol. 2014 Oct;49(10):1245-54. doi: 10.3109/00365521.2014.946083. Epub 2014 Aug 15.

Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands.

Author information

1
Department of Gastroenterology and Hepatology, VU University Medical Center , Amsterdam , The Netherlands.

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.

KEYWORDS:

autoimmune hepatitis; cirrhosis; diagnosis; diagnostic scoring systems; epidemiology; hepatocellular carcinoma; prevalence; transplantation

PMID:
25123213
DOI:
10.3109/00365521.2014.946083
[Indexed for MEDLINE]

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