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Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study.
J Hepatol. 2014 Mar; 60(3):612-7.JH

Abstract

BACKGROUND & AIMS

Population-based studies of the clinical course of autoimmune hepatitis are scarce. We conducted a nationwide study of incidence, prevalence, prognosis, and causes of death of autoimmune hepatitis in Denmark.

METHODS

From nationwide healthcare registries we identified all Danish citizens diagnosed with autoimmune hepatitis in 1994-2012 and their liver biopsy data. We followed patients through January 2013 and examined age-standardized incidence and prevalence, mortality, prognostic factors, risk of hepatocellular carcinoma, and causes of death. We used Cox regression to compare patients' mortality relative to a gender- and age-matched general population sample.

RESULTS

We included 1721 autoimmune hepatitis patients. The incidence rate was 1.68 (95% confidence interval 1.60 to 1.76) per 100,000 population per year, and it doubled during the study period. Of the 1318 patients who were biopsied at diagnosis, 28.3% had cirrhosis. The 10-year cumulative risk of hepatocellular carcinoma was 0.7% (95% confidence interval 0.3 to 1.5). Male gender and cirrhosis were associated with high mortality and development of hepatocellular carcinoma. In the first year after diagnosis, patients with autoimmune hepatitis had six-fold higher mortality than the general population; later, their mortality remained two-fold higher. Their 10-year cumulative mortality was 26.4% (95% confidence interval 23.7 to 29.1). 38.6% of deaths were liver-related including 3.6% from hepatocellular carcinoma.

CONCLUSIONS

This nationwide population-based study of autoimmune hepatitis showed that the incidence increased during 1994-2012, and that the disease remains associated with a high mortality, particularly in the first year after diagnosis. Male gender and cirrhosis were adverse prognostic factors.

Authors+Show Affiliations

Department of Hepatology and Gastroenterology, Aarhus University Hospital, Denmark. Electronic address: groenbaek.lisbet@gmail.com.Department of Hepatology and Gastroenterology, Aarhus University Hospital, Denmark.Department of Hepatology and Gastroenterology, Aarhus University Hospital, Denmark; Department of Clinical Epidemiology, Aarhus University Hospital, Denmark.

Pub Type(s)

Journal Article

Language

eng

PubMed ID

24326217

Citation

Grønbæk, Lisbet, et al. "Autoimmune Hepatitis in Denmark: Incidence, Prevalence, Prognosis, and Causes of Death. a Nationwide Registry-based Cohort Study." Journal of Hepatology, vol. 60, no. 3, 2014, pp. 612-7.
Grønbæk L, Vilstrup H, Jepsen P. Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. J Hepatol. 2014;60(3):612-7.
Grønbæk, L., Vilstrup, H., & Jepsen, P. (2014). Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. Journal of Hepatology, 60(3), 612-7. https://doi.org/10.1016/j.jhep.2013.10.020
Grønbæk L, Vilstrup H, Jepsen P. Autoimmune Hepatitis in Denmark: Incidence, Prevalence, Prognosis, and Causes of Death. a Nationwide Registry-based Cohort Study. J Hepatol. 2014;60(3):612-7. PubMed PMID: 24326217.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Autoimmune hepatitis in Denmark: incidence, prevalence, prognosis, and causes of death. A nationwide registry-based cohort study. AU - Grønbæk,Lisbet, AU - Vilstrup,Hendrik, AU - Jepsen,Peter, Y1 - 2013/10/26/ PY - 2013/04/30/received PY - 2013/10/10/revised PY - 2013/10/14/accepted PY - 2013/12/12/entrez PY - 2013/12/12/pubmed PY - 2014/12/15/medline KW - AIH KW - Autoimmune hepatitis KW - Autoimmune liver disease KW - CI KW - Causes of death KW - Epidemiology KW - HCC KW - Hepatocellular carcinoma KW - ICD-10 KW - ICD-8 KW - Incidence KW - International Classification of Diseases, 10th edition KW - International Classification of Diseases, 8th edition KW - Prevalence KW - Prognosis KW - confidence interval KW - hepatocellular carcinoma SP - 612 EP - 7 JF - Journal of hepatology JO - J Hepatol VL - 60 IS - 3 N2 - BACKGROUND & AIMS: Population-based studies of the clinical course of autoimmune hepatitis are scarce. We conducted a nationwide study of incidence, prevalence, prognosis, and causes of death of autoimmune hepatitis in Denmark. METHODS: From nationwide healthcare registries we identified all Danish citizens diagnosed with autoimmune hepatitis in 1994-2012 and their liver biopsy data. We followed patients through January 2013 and examined age-standardized incidence and prevalence, mortality, prognostic factors, risk of hepatocellular carcinoma, and causes of death. We used Cox regression to compare patients' mortality relative to a gender- and age-matched general population sample. RESULTS: We included 1721 autoimmune hepatitis patients. The incidence rate was 1.68 (95% confidence interval 1.60 to 1.76) per 100,000 population per year, and it doubled during the study period. Of the 1318 patients who were biopsied at diagnosis, 28.3% had cirrhosis. The 10-year cumulative risk of hepatocellular carcinoma was 0.7% (95% confidence interval 0.3 to 1.5). Male gender and cirrhosis were associated with high mortality and development of hepatocellular carcinoma. In the first year after diagnosis, patients with autoimmune hepatitis had six-fold higher mortality than the general population; later, their mortality remained two-fold higher. Their 10-year cumulative mortality was 26.4% (95% confidence interval 23.7 to 29.1). 38.6% of deaths were liver-related including 3.6% from hepatocellular carcinoma. CONCLUSIONS: This nationwide population-based study of autoimmune hepatitis showed that the incidence increased during 1994-2012, and that the disease remains associated with a high mortality, particularly in the first year after diagnosis. Male gender and cirrhosis were adverse prognostic factors. SN - 1600-0641 UR - https://www.unboundmedicine.com/medline/citation/24326217/Autoimmune_hepatitis_in_Denmark:_incidence_prevalence_prognosis_and_causes_of_death__A_nationwide_registry_based_cohort_study_ DB - PRIME DP - Unbound Medicine ER -