“…Regarding AIH-PBC overlap, several studies have shown lower response to ursodeoxicholic acid (UDCA), higher risk of disease progression and decompensation, when compared to classical PBC (139) . More than three hundred patients with overlap syndromes have been reported in the literature, including case reports and cohorts of patients, due to the concurrent presence of patients with AIH with cholestatic or mixed biochemical profile, IgM elevation, bile duct lesions or granulomas at histology, absence of serological markers or alternatively presence of AMA or anti-M2, absence of response to IS, association with inflammatory bowel disease and cholangiographic findings typical of PSC, as well as PBC subjects with disproportionate elevation of aminotransferases, IgG elevation, presence of high-titer autoantibodies with the exception of AMA, prominent portal and lobular inflammation and response to IS; and large-duct or small-duct PSC patients with disproportionate elevation of aminotransferases, IgG elevation, presence of high-titer autoantibodies, prominent portal and/or lobular inflammation, granulomas and response to IS (8,(175)(176)(177)(178) . Several papers have been published with the intention to better characterize AIH-PBC overlap using several diagnostic descriptive criteria and scores, including Paris criteria ( Figure 3) (178) and the original, revised and simplified criteria of the International Autoimmune Hepatitis Study Group (IAIHSG) (176) .…”