There are three investigations upon which the diagnosis of PBC is based. The presence of two of these findings is needed to make the diagnosis of PBC in practice and to trigger treatment. Liver biopsy is now only rarely done in PBC meaning that most patients are diagnosed based on the combination of antibodies and liver function tests.

There is no cure for PBC at present and the standard approach to the treatment of the disease is to reduce the impact of toxic bile acids that build up in the liver because the reduced bile flow. Standard practice is to use a “step up” approach.