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Primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) have been identified in the same patients — a coexisting state known as overlap syndrome. Evidence shows that patients with PBC benefit from ursodiol therapy, whereas those with AIH receive immunosuppressive therapy; however, we know very little about the natural history, optimal treatment strategy, and long-term outcomes of PBC-AIH overlap syndrome.
A cohort of 137 PBC patients (mean age, 61; 87% women) from a previous Mayo Clinic study were evaluated for AIH using a revised scoring system for overlap syndrome that was proposed by the International Autoimmune Hepatitis Group (J Hepatol 1999; 31:929). Based on these criteria, probable overlap syndrome was diagnosed in 26 patients…