Abstract
Testing for liver-related autoantibodies should be included in the workup of patients with hepatitis or cholestasis of unknown origin. Although most of these autoantibodies are not disease specific, their determination is a prerequisite to diagnose autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), and they are components of the diagnostic scoring system in these diseases. In primary sclerosing cholangitis (PSC), on the other hand, autoantibodies are frequently present but play a minor role in establishing the diagnosis. In PSC, however, data on antibodies suggest a link between disease pathogenesis and the intestinal microbiota. This review will focus on practical aspects of antibody testing in the three major autoimmune liver diseases AIH, PBC, and PSC.
Highlights
Testing for liver-related autoantibodies should be included in the workup of patients with hepatitis or cholestasis of unknown origin
There are hints at a relevant role for a humoral immune response in autoimmune hepatitis (AIH) pathogenesis: autoantibodies can frequently be detected, plasma cells are enriched in hepatic infiltrates and immunoglobulin G (IgG)/gammaglobulins serve as a diagnostic and disease activity markers
For the diagnostic workup of autoimmune liver diseases, anti-soluble liver antigen/ liver–pancreas antibodies should already initially be tested for by enzyme-linked immunosorbent assays (ELISA) or immunoblot since these autoantibodies cannot be detected by immunofluorescence testing (IFT) and have high specificity for AIH
Summary
Testing for liver-related autoantibodies should be included in the workup of patients with hepatitis or cholestasis of unknown origin Most of these autoantibodies are not disease specific, their determination is a prerequisite to diagnose autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), and they are components of the diagnostic scoring system in these diseases [1, 2] (Table 1). The following review will focus on practical aspects of antibody testing in the three major autoimmune liver diseases AIH, PBC, and PSC
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