Possible Role of the HMGB1 and RAGE inflammatory pathway in primary sclerosing cholangitis

•This study aimed to assess the impact of proinflammatory HMGB1 and one of its receptors RAGE in PSC pathogenesis.•Biliary HMGB1 was significantly elevated in PSC patients.•A gain-of-function single nucleotide polymorphism (SNP) in RAGE was associated with development of dominant stenosis.•PSC patients with the mutant allele of the RAGE SNP had significantly reduced transplantation-free survival.