| Autoimmune liver diseases (AILDs) encompass a spectrum of chronic inflammatory hepatobiliary diseases including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC), and IgG4-associated cholangitis, as well as syndromes with overlapping features. This lecture will address AIH and PBC. AIH and PBC are rare diseases with limited epidemiological data. AILD incidence is estimated to be 1/100,000 people, but may be lower in Asia than in Western countries. Reported AIH prevalences per 100,000 people are 4 in Singapore, 4.8 in South Korea, 8 in Australia, and 23.4 in Japan, while prevalence ranges between 10.7-23.9 in Western populations. Despite 2 established scoring systems, AIH diagnosis remains unclear especially in cases with acute and severe presentation, nonalcoholic fatty liver, or overlapping syndromes. First line therapy is prednisolone with or without azathioprine, resulting in 80% complete response rate. Commonly used second line therapy includes mycophenolate or cyclosporines.Reported PBC prevalences per 100,000 people are 1.9 in Australia, 4.75 in South Korea, 5 in Japan, 49 in China, 15 in Sweden, 24 in UK, and 40.2 in Minnesota USA. PBC diagnosis is straightforward compared to AIH, mostly accompanied by presence of anti-mitochondrial antibodies. First line therapy is ursodeoxycholic acid (UDCA; 13-15 mg/kg/day). For incomplete responders (up to 40%), obeticholic acid or bezafibrate (or fenofibrate) is combined with UDCA, however, obeticholic acid bezafibrates are not approved in many Asian countries. AILD can progress to cirrhosis and then hepatocellular carcinoma (HCC). HCC incidence is 3.06/1000 and 3/1000 patient-years in AIH and PBC, respectively. Risk factors for HCC development in AIH are older age, male sex, baseline cirrhosis, AIH relapses, and alcohol use. Risk factors in PBC are male sex, advanced liver disease, and biochemical non-response. Liver transplantation (LT) is indicated for AILD patients with end-stage liver disease with 5 year survival more than 80%. AILD recurs in about 30% of LT patients and can newly arise in LT recipients whose original indications for LT were not AILD. Both cases respond well to standard AIH therapies. In conclusion, there is a lack of data, awareness (with subsequent delayed diagnosis), and 2nd line therapeutics concerning AILDs in Asia. Cooperation among Asian hepatology societies is needed to address these concerns. |
