Autoimmune liver diseases are chronic inflammatory hepatobiliary disorders that when classically defined encompass three distinctive clinical presentations; primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH). Meaningful changes in disease epidemiology are reported, with increasing incidence and prevalence of AIH and PSC in Europe, and rising prevalence of PBC across Europe, North America and the Asia-Pacific region. However, there appears to be very significant global variation with contemporary incidence rates of disease per 100 000 ranging from 0.84 to 2.75 for PBC, 0.1 to 4.39 for PSC and 0.4 to 2.39 for AIH. Prevalence corresponds, and per 100 000 estimates for PBC range from 1.91 to 40.2, for PSC between 0.78 and 31.7 and for AIH from 4.8 to 42.9. Population-based studies and multicentre observational cohort series provide improved understanding of the clinical course that patients experience, highlighting variations in presenting phenotypes geographically and temporally. Collectively, while autoimmune liver diseases are rare, the clinical burden is disproportionately high relative to population incidence and prevalence. Age, sex and race also impact clinical outcomes, and patient morbidity and mortality are reflected by high need for gastroenterology, hepatology and organ transplant services.
- primary biliary cirrhosis
- primary sclerosing cholangitis
- autoimmune hepatitis
- autoimmune liver disease
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Correction notice This article has been corrected since it published Online First. Figure 1 has been replaced.
Contributors Original idea: GH, first draft: PT, revisions and editing: PT and GH. Guarantor: PT.
Funding Palak J. Trivedi receives institutional salary support from the National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre (BRC). This paper presents independent research supported by the Birmingham NIHR BRC based at the University Hospitals Birmingham National Health Service Foundation Trust and the University of Birmingham. The views expressed are those of the author(s) and not necessarily those of the National Health Service, the NIHR, or the Department of Health. Dr. Trivedi has received grant support from the Wellcome Trust, the Medical Research Foundation, LifeArc, GSK, Guts UK, PSC Support, Intercept Pharma, Dr. Falk Pharma, Gilead sciences, and Bristol Myers Squibb. He has also received speaker fees from Intercept and Dr Falk, and advisory board / consultancy fees from Intercept, Dr. Falk and GSK. GMH has consulted for Cymabay, Genfit, GSK, Falk, Intercept, Pliant and Roche. GMH is supported by the Lily and Terry Horner Chair in Autoimmune Liver Disease Research.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Provenance and peer review Commissioned; externally peer reviewed.
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