2018 Volume 32 Issue 2 Pages 233-240
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease, but pathophysiology of this disease has not been clarified yet. However, recent studies on disease susceptible genes and immunological mechanisms of cholangiocyte destruction are unveiling the cause of this disease. A new classification of histopathological stages and activities was established, and the so-called 'PBC/AIH overlap syndrome' has come to be classified as a PBC with high activity of hepatitis. A new class medicine, obeticholic acid, has been used in the United States since 2016, but clinical usability of this medicine is unknown because of frequent side-effect of pruritus and tremendously high cost. In this situation, the usefulness of bezafibrate that has been used as a second-line medicine for more than 10 years in Japan has come to be recognized recently in western countries. In addition, clinical trials on immunotherapies are also ongoing. New scoring systems to predict outcomes of patients with PBC (GLOBE and UK-PBC scores) were established recently and the calculators are freely available on website.
