| Primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) have collectively been recognized as autoimmune liver diseases. The incidence of the autoimmune liver diseases is around 1-2 per 100,000 population per year for each disease. In Taiwan, the annual incidence of AIH was reported to be 0.52/105. The female/male sex ratios and the median age were 15/7~ 37/11 and 58~64 yrs for adult AIH, as well as 5/4 and 14 yrs for pediatric AIH. The antinuclear antibodies (ANA) positive rate was 98% among the AIH patients. Although the prevalence of jaundice and cirrhosis may be as high as 59 % and 35 %, respectively, most patients responded well to a lower dose of prednisolone than reported in the Western literature. By contrast, the prevalence or incidence of PBC in Taiwan remained uncertain. The mean or median age of the enrolled PBC patients in studies based in Taiwan ranged from 50.3 to 57.4 years old, and the female/male ratios ranged from 74/22 to 27/3. The anti-mitochondrial antibody (AMA) -positive rates ranged from 88.5 to 94%, and the ANA-positive rates ranged from 48 to 73%, while elevated IgM were noted in 88~89% of the PBC patients. Although the AMA-positive and AMA-negative PBC patients had similar clinical manifestations and prognosis, the former had higher elevated IgM rates but lower ANA-positive rates than the latter. Levels of albumin, creatinine, bilirubin and prothrombin time, as well as scores of Mayo risk, Model For End-Stage Liver Disease, and Child-Pugh, and histology and aspartate aminotransferase/alanine transaminase ratios were regarded to be associated with the outcomes of PBC patients. The prognosis was good for PBC patients underwent liver transplantation, as the 5-year survival rate was up to 89.2%. Being an area hyperendemic for chronic hepatitis B (CHB), the prevalences of CHB in Taiwan is around 17%. However, the prevalence of CHB in PBC patients was as low as 0~3.8% and the associated basis remained inconclusive. Besides, the co-existence of other diseases including chronic hepatitis C (CHC) in PBC patients leads to a poorer intra-hepatic and extra-hepatic prognosis than that in patients with PBC alone. The current status regarding the interactions among PBC, CHB and CHC in Taiwan will be elucidated in a tertiary care centre-based study with enrollment of 472 AMA-positive PBC patients. |
