Retrospective analysis of autoimmune hepatitis-primary biliary cirrhosis overlap syndrome in Korea: characteristics, treatments, and outcomes.

摘要: Background/aims Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) (AIH-PBC overlap syndrome) is a rare disease that has not been clearly characterized in Korean patients. This study investigated the clinical features AIH-PBC compared with those AIH PBC alone. Methods retrospective cohort included 158 consecutive patients who were diagnosed as (n=61), (n=81), or (n=9) based on Paris International Autoimmune Hepatitis Group (IAIHG) criteria from 2001 to 2011 Korea. We these three groups retrospectively, including their biochemical characteristics, treatments, responses, outcomes. Results The exhibited characteristics both PBC, showed similar response ursodeoxycholic acid (UDCA) monotherapy for However, UDCA steroid combination therapy was worse than steroid-based (P=0.024). Liver developed more rapidly group (P=0.013), but there no difference between rates developing hepatic decompensation did differ significantly groups. Conclusions faster cirrhotic progression