Primary biliary cirrhosis (PBC) is a chronic, organ-specific, autoimmune liver disease characterized by progressive cholestasis, eventually leading to cirrhosis. Several lines of evidence have revealed a crucial role of adaptive as well as innate immune responses in the etiopathogenesis of PBC, and more recently, the biology of bile duct cells and genome-wide association studies (GWAS) demonstrated several key molecules and pathways in this enigmatic disease. Although ursodeoxycholic acid (UDCA) has been the only approved drug for PBC with clinical evidences for improvement of long-term outcomes, a substantial population have suboptimal responses to UDCA, resulting in unfavorable outcomes. In this regard, second-line treatment for patients refractory to UDCA is strongly awaited. In Japan, bezafibrate (BF) has been frequently used for this purpose, yet recent clinical trials failed to clearly demonstrate clinical efficacy of BF. Novel pharmacotherapies targeted to key molecules and pathways in PBC are upcoming. Finally, we sincerely call on all members of the Japan Society of Hepatology to use from this moment on the name "primary biliary cholangitis" for the disease known by its abbreviation PBC, in keeping with a very recent global agreement.
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