The Hepatitic/Cholestatic "Overlap" Syndrome: An Italian Experience

Background: Patients with hepatitic and cholestatic autoimmune liver disease (overlap syndrome) represent a diagnostic and therapeutic challenge. Aim: To evaluate the prevalence of the hepatitic/cholestatic overlap in a large series of consecutive patients with cholestatic autoimmune liver disease. Methods: We re-evaluated the diagnosis of 235 patients with autoimmune liver disease, including 70 with type 1 autoimmune hepatitis (AIH), 142 with primary biliary cirrhosis (PBC), and 23 with primary sclerosing cholangitis (PSC), using the revised International Autoimmune Hepatitis Group (IAIHG) scoring system. Anti-mitochondrial, anti-nuclear, anti-smooth muscle, anti-liver kidney microsomal type 1, anti-liver cytosol type 1, perinuclear anti-neutrophil nuclear and anti-soluble liver antigen antibodies were evaluated in each patient. Results: Ten patients (3 with a previous diagnosis of PBC and 7 of PSC) scored as probable or definite AIH. These patients did not have a specific autoantibody profile. C...