Intended for healthcare professionals

Clinical Review

Autoimmune liver disease for the non-specialist

BMJ 2009; 339 doi: (Published 08 September 2009) Cite this as: BMJ 2009;339:b3305
  1. Sofie Decock, hepatologist1,
  2. Phyllis McGee, nurse practitioner with interest in autoimmune liver disease2,
  3. Gideon M Hirschfield, assistant professor of medicine2
  1. 1AZ Sint-Lucas, Belgium
  2. 2The Liver Centre, Toronto Western Hospital, Toronto, ON, Canada, M5T 2S8
  1. Correspondence to: Dr Gideon M Hirschfield, Toronto Western Hospital, 399 Bathurst Street, 6B Fell Pavilion, Toronto, ON, M5T 2S8 gideon.hirschfield{at}

    Summary points

    • Autoimmune liver diseases are chronic diseases, each with a long natural course

    • Disease need not be symptomatic, but symptoms when present can be challenging to manage

    • Autoimmune hepatitis is a parenchymal disease, whereas primary biliary cirrhosis and primary sclerosing cholangitis are biliary diseases

    • Medical management of primary biliary cirrhosis (with ursodeoxycholic acid) and autoimmune hepatitis (with prednisolone and azathioprine) is very successful and transplantation is rarely needed

    • Primary sclerosing cholangitis lacks effective medical interventions, but transplantation when indicated is highly successful

    Autoimmune hepatitis, primary biliary cirrhosis, and sclerosing cholangitis represent perhaps 5% of all liver diseases, although no registries exist to estimate the true prevalence. They are presumed autoimmune conditions, usually considered as a diagnosis after viral, metabolic, and drug induced liver injuries have been excluded. The combination of medical and surgical treatments means that, if appropriately diagnosed and managed, these diseases overall have an excellent prognosis. We review these conditions for non-specialists and refer to available guidelines and clinical trial data.1 2 3

    What are the epidemiological and clinical features of autoimmune liver disease?

    Autoimmune liver diseases are chronic, slowly progressive, inflammatory liver diseases that may have overlapping features.4 (fig 1, table 1).

    Fig 1 Characteristic histological and radiological features of autoimmune liver diseases

    View this table:
    Table 1

     Summary features of autoimmune liver disease

    Autoimmune hepatitis

    Autoimmune hepatitis is a relapsing idiopathic hepatitis, encountered more often in women than men across all ages and ethnicities. Ascertainment biases limit available epidemiology; a Swedish study put the annual incidence as 8.5 per 1 000 000 population and point prevalence as 107 per 1 000 000.5 Patients present clinically with arthralgias and fatigue if symptomatic, and a third of patients present with cirrhosis.w1 Raised liver enzymes (transaminases) characterise initial laboratory abnormalities.

    Primary biliary cirrhosis

    Primary biliary cirrhosis is a slowly progressive, chronic cholestatic disease affecting primarily middle aged women (female:male ratio 9 to 1) and is characterised by …

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