Making Sense of Autoantibodies in Cholestatic Liver Diseases

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the most common chronic cholestatic liver diseases (CLD) in adults, and are associated with immune mechanisms. PBC is considered a model autoimmune disease, and more than 90% of patients present very specific autoantibodies against mitochondrial antigens. Whether PSC should be considered an autoimmune, or merely immune-mediated disease, is still under debate. This review addresses the clinical relevance of autoantibodies in OLD and their pathogenic mechanisms, and it illustrates the technology available for appropriate autoantibody detection.