Epidemiology and clinical characteristics of autoimmune hepatitis in the Netherlands

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

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.

Publication types

  • Multicenter Study

MeSH terms

  • Adolescent
  • Adult
  • African Continental Ancestry Group / statistics & numerical data
  • Age Factors
  • Aged
  • Aged, 80 and over
  • Alanine Transaminase / blood
  • Alkaline Phosphatase / blood
  • Anti-Inflammatory Agents / therapeutic use
  • Antibodies, Antinuclear / blood
  • Asian Continental Ancestry Group / statistics & numerical data
  • Carcinoma, Hepatocellular / epidemiology*
  • Child
  • Child, Preschool
  • European Continental Ancestry Group / statistics & numerical data
  • Fatigue / etiology
  • Female
  • Hepatitis, Autoimmune / diagnosis
  • Hepatitis, Autoimmune / drug therapy
  • Hepatitis, Autoimmune / epidemiology*
  • Hepatitis, Autoimmune / genetics
  • Humans
  • Immunoglobulin G / blood
  • Immunosuppressive Agents / therapeutic use
  • Incidence
  • Jaundice / etiology
  • Liver Cirrhosis / epidemiology
  • Liver Neoplasms / epidemiology*
  • Male
  • Middle Aged
  • Netherlands / epidemiology
  • Prevalence
  • Retrospective Studies
  • Sex Factors
  • South America / ethnology
  • Surveys and Questionnaires
  • Young Adult

Substances

  • Anti-Inflammatory Agents
  • Antibodies, Antinuclear
  • Immunoglobulin G
  • Immunosuppressive Agents
  • Alanine Transaminase
  • Alkaline Phosphatase