Although anti-PR3 was previously reported to be present in a higher proportion of PSC patients compared to other autoimmune liver conditions (Stinton et al., 2014), its lack of specificity for PSC (Mulder et al., 1993; Weiner and Segelmark, 2016) limits its use in the diagnosis of PSC. This evidence concerns the gene PRTN3 and pancreatic serous cystadenoma.