This overlap syndrome is characterized by the coexistence of clinical, biochemical, or serological features of autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC). In about 5% of patients with a primary diagnosis of AIH, laboratory signs and clinical symptoms of PBC exist. On the other hand, 19% of patients with a primary diagnosis of PBC also have signs of AIH. The overlap of PBC and AIH is characterized by the presence of ANAs in 67% and antibodies against SMAs in 67%. Because patients with an overlap of PBC and AIH respond to corticosteroid treatment equally well as patients with primary AIH, the identification of this variant group by autoantibody characterization is required and contributes to the establishment of an efficacious therapeutic strategy [74, 75].
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