Interestingly, overexpression of DMBT1 was shown in primary sclerosing cholangitis as well as in hepatolithiasis (Bisgaard et al. 2002; Sasaki et al. 2003), two common risk factors for the occurrence of LD-iCCA (Aishima and Oda 2015). This evidence concerns the gene PRRT2 and sclerosing cholangitis.