Abstract
Autoimmune hepatitis, primary biliary cholangitis, and primary sclerosing cholangitis are immune-mediated chronic liver diseases of uncertain cause that have a global distribution and highly variable occurrence. The goals of this review are to describe the epidemiological studies that have identified the populations at risk, estimated the incidence and prevalence of each disease, suggested environmental and genetic bases for their occurrence, and indicated trends that should direct the allocation of healthcare resources and investigational efforts. Population-based epidemiological studies have described patterns of susceptibility for each liver disease that reflect predilections for certain age groups, gender, geographical regions, and ethnic background. Familial studies and genetic analyses have implicated a genetic predisposition for each disease, and population-based studies have suggested associations with triggering agents, including pollutants, xenobiotics, viruses, bacteria, and the intestinal microbiome. Variations in prevalence between ethnic groups within regions or between countries may reflect differences in early diagnosis, management, and outcome, and the increasing incidence of these diseases in certain regions and ethnic groups may help identify pivotal etiological factors that might be modified. Population-based epidemiological studies are lacking in China, India, and developing countries, and they are needed to complete the global perspective of these diseases and their consequences. In conclusion, autoimmune hepatitis and the immune-mediated cholangiopathies are rare, but they constitute a global healthcare burden that is increasing in certain geographical regions and ethnic groups. Populations at risk and susceptibility factors must continue to be characterized, and interventions must be tailored to meet individual and regional needs.
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Acknowledgments
This review did not receive financial support from a funding agency or institution, and Albert J. Czaja, MD has no conflict of interests to declare.
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Table of Landmark Literature
Table of Landmark Literature
Study title and authors | Study design | Summary results | Main limitations |
|---|---|---|---|
Ngu JH et al. J Gastroenterol Hepatol 2010;25:1681–86 | Population-based study of AIH in Canterbury, New Zealand | • Peak age in sixth decade • Ethnic-specificity | • Tertiary referral center over-representative |
Gronbaek L, et al. J Hepatol 2014;60:612–17 | Population-based study of AIH using Danish National Patient Registry | • Cirrhosis in 28% • Incidence increasing • Prognosis improving | • Uncertain uniformity of diagnostic criteria |
Van Gerven NM, et al. Scand J Gastroenterol 2014;49:1245–54 | Population-based study of AIH in the Netherlands | • Concurrent immune diseases in 26% | • Incomplete datasets • Diagnostic uncertainties |
Sood S, et al. Gastroenterology 2004;127:470–5 | Population-based studies of PBC in Victoria, Australia | • Victorians protected • Absent environmental trigger possible | • Inconsistent case finding methods used |
Myers RP, et al. Hepatology 2009;50:1884–92 | Population-based study of PBC in Calgary, Canada | • Increasing prevalence • Need for better therapy | • Risk factors unavailable • Incomplete datasets |
Gershwin ME, et al. Hepatology 2005;42:1194–1202 | Large case-controlled interview-based study of PBC | • Risk factors of family history, UTIs, past smoking, and HRT | • Patients highly selected • Dependent on patient’s recall and understanding |
Bambha K, et al. Gastroenterology 2003;125:1364–69 | Population-based study of PSC in U.S. community | • Male predominance • 73% of PSC with IBD • Poor overall survival | • Uncertain case discovery |
Molodecky NA, et al. Hepatology 2011;53:1590–99 | Meta-analysis of 8 mainly population-based studies of PSC | • Incidence increasing | • Small number of studies • No prevalence data |
Boonstra K, et al. Hepatology 2013;58:2045–55 | Population-based study of PSC in the Netherlands | • Lower survival in tertiary referral centers • CRC increased | • Incomplete datasets • Uncertain case discovery |
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Czaja, A.J. (2019). Autoimmune Hepatitis and Immune-Mediated Cholestatic Liver Diseases. In: Wong, R., Gish, R. (eds) Clinical Epidemiology of Chronic Liver Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-94355-8_16
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