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International Session (Symposium)1 (JSH・JSGE・JGES)
Thu. October 12th   9:40 - 12:00   Room 11: Fukuoka International Congress Center 502+503
Overview of Autoimmune liver disease
C. Efe
Batman State Hospital
Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC, formerly primary biliary cirrhosis) are two main autoimmune liver diseases. They are relatively uncommon cause of chronic liver disease in Asia but, recent studies showed that prevalence of autoimmune liver diseases is increasing in worldwide as well in Asia. Therefore, early diagnosis and effective management of these diseases is essential to prevent liver related complications and death. The simplified criteria which are based on circulating auto-antibodies, IgG levels, absence of viral hepatitis and histological findings have been proposed for AIH diagnosis. Predniso(lo)ne alone or in combination with azathioprine, is the standard therapy of AIH. Mycophenolate mofetil, tacrolimus and infliximab are other rescue therapies, in up to 20% patients who do not respond or intolerant to standard therapy.

The diagnosis of PBC is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis, anti-mitochondrial antibodies and suggestive histological findings. Ursodeoxycholic acid (UDCA) is the first approved therapy for PBC. This treatment slows the progress of the disease, but approximatively 30-40% of patients fail to respond to UDCA. Recently, Obeticholic acid (OCA), a Farnesoid X Receptor agonist, has been approved for patients who are inadequate responders to UDCA.

A smaller group of PBC patients may present or develop AIH features. The term ‘overlap syndrome’ describes this rare clinical condition, although there are no well-established diagnostic criteria. These patients should be treated based on liver histology. Patients who have moderate interface hepatitis can be initially treated by UDCA alone while those with severe hepatitis require additional immunosuppression.
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