Primary sclerosing cholangitis articles from across Nature Portfolio

CD8 + T cells play an important role in primary biliary cholangitis (PBC). In this study, the authors demonstrate that PD-1-expressing CD8+ liver-resident T cells are pathogenic in murine PBC and that targeting these cells using a chimaeric antigen receptor reduces disease severity.

The association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) has been known for decades, but mechanisms of gut-liver crosstalk are incompletely understood. Here, the authors show a colitis-triggered protective circuit suppressing cholestatic liver disease which encourages multi-organ treatment strategies for PSC.

Patients with primary sclerosing cholangitis (PSC) harboring Klebsiella pneumoniae (Kp) exhibit poor clinical outcomes. Here, the authors show that administration of a phage cocktail targeting PSC-derived Kp reduces bacterial burden in Kp-colonized mice and alleviates liver injury.

Primary sclerosing cholangitis (PSC) is closely associated with inflammatory bowel disease (IBD) and potentially provides unique insights into the gut–liver axis. This Review explores these links and provides an overview of the gut microbiome in PSC, including PSC–IBD, exploring related hypotheses of disease mechanisms.

Autoimmune pancreatitis and IgG4-related cholangitis are frequent manifestations of IgG4-related disease (IgG4-RD). Here, Löhr and colleagues present current understanding of the pathophysiology of IgG4-RD and describe all IgG4-RD manifestations in the digestive tract. Diagnosis, prognosis and treatment of IgG4-RD are also discussed.

Klebsiella pneumoniae isolated from patients with primary sclerosing cholangitis was found to cause intestinal barrier dysfunction resulting in T-helper-17-cell-mediated hepatobiliary injury, providing evidence for specific gut-derived, pore-forming pathogens as triggers for immune-mediated liver disease.

A new study of a fibroblast growth factor 19 analogue in patients with primary sclerosing cholangitis (PSC) provides provocative results. The data challenge alkaline phosphatase levels as the appropriate surrogate end point in PSC trials and highlight alternatives, urging efforts to identify better clinical end points for this disease.

Although a colonoscopy for the evaluation of IBD and the surveillance of colitis-associated neoplasia should be performed at the time of the diagnosis of primary sclerosing cholangitis (PSC), the results from a new study suggest that screening for PSC with magnetic resonance cholangiography in patients with long-term IBD might be needed.