Efe C, Torgutalp M, Henriksson I, et al. - This retrospective review of 1,554 patients with primary biliary cholangitis (PBC) from 20 international centers was carried out to determine the prevalence as well as the clinical importance of extrahepatic autoimmune diseases (EHAIDs) in a large cohort of patients with PBC. In 440 (28.3%) patients with PBC, 35 different EHAIDs were diagnosed. More often, female gender and seropositivity for anti‐mitochondrial antibodies and antinuclear antibodies and/or smooth muscle antibodies were noted in cases with EHAIDs. Significantly lower levels of alkaline phosphatase, aspartate aminotransferase, and total bilirubin were seen at presentation in patients with EHAIDs. Overall, a frequent diagnosis of EHAIDs in patients with PBC was corroborated in this study. The clinical phenotype of PBC at presentation may be impacted by the presence of EHAIDs, but PBC outcome is not.