Primary biliary cirrhosis articles from across Nature Portfolio

Primary biliary cholangitis is a rare, chronic immune-mediated liver disease triggered by environmental exposures in genetically susceptible individuals. Here, the authors investigate the functional mechanism underlying the association of 19p13.3 variants with primary biliary cholangitis.

The aetiology of primary biliary cholangitis (PBC) remains unclear. Here, the authors find that the numbers of DUOX2⁺ ACE2 ⁺ small cholangiocytes in human and mouse livers are inversely associated with disease severity, and present data indicating that they may be the target of polymeric immunoglobulin receptor (pIgR) -mediated humoral responses, suggesting that preservation of these cells and targeting anti-pIgR autoantibodies may be valuable strategies for therapeutic interventions in PBC.

In 2016, obeticholic acid became the first new licensed therapy for primary biliary cholangitis in >20 years. This therapeutic came at a time of improved disease understanding from biliary and immunological mechanistic insights.

What is in a name; and why does the nomenclature of one disease, primary biliary cirrhosis, cause such contention that a proposed name change by those deeply committed to the advancement of its understanding and management stimulate huge debate?