• Akira Honda, Atsushi Tanaka, Tetsuji Kaneko, Atsumasa Komori, Masanori Abe, Mie Inao, Tadashi Namisaki, Naoaki Hashimoto, Kazuhito Kawata, Atsushi Takahashi, Masashi Ninomiya, Jong-Hon Kang, Mie Arakawa, Satoshi Yamagiwa, Satoru Joshita, Takeji Umemura, Ken Sato, Akira Kaneko, Kentaro Kikuchi, Jun Itakura, Takako Nomura, Keisuke Kakisaka, Hideki Fujii, Norifumi Kawada, Yasuhiro Takikawa, Tsutomu Masaki, Hiromasa Ohira, Satoshi Mochida, Hitoshi Yoshiji, Satoshi Iimuro, Yasushi Matsuzaki, Hajime Takikawa, and Japan PBC Study Group.
• Division of Gastroenterology and Hepatology, Department of Internal Medicine, Tokyo Medical University Ibaraki Medical Center, Ibaraki, Japan.
• Hepatology. 2019 Dec 1; 70 (6): 2035-2046.

AbstractIn Japan, bezafibrate (BF) is a second-line agent for primary biliary cholangitis (PBC) that is refractory to ursodeoxycholic acid (UDCA) treatment. From a retrospective cohort (n = 873) from the Japan PBC Study Group, we enrolled 118 patients who had received UDCA monotherapy for at least 1 year followed by combination therapy with UDCA+BF for at least 1 year. GLOBE and UK-PBC scores after UDCA monotherapy (i.e., immediately before UDCA+BF combination therapy) were compared with those after 1 year of UDCA+BF combination therapy. The real outcomes of enrolled patients estimated by Kaplan-Meier analysis were compared with the predicted outcomes calculated using GLOBE and UK-PBC scores. In addition, the hazard ratio of BF treatment was calculated using propensity score analysis. The mean GLOBE score before the combination therapy was 0.504 ± 0.080, which improved significantly to 0.115 ± 0.085 (P < 0.0001) after 1 year of combination therapy. The real liver transplant-free survival of enrolled patients was significantly better than that predicted by GLOBE score before introducing BF. Combination therapy did not significantly improve the real rates of liver transplantation or liver-related death compared with those predicted by UK-PBC risk score before introducing BF, but the predicted risk was significantly reduced by the addition of BF (P < 0.0001). Cox regression analysis with inverse probability of treatment weighting showed that the addition of BF significantly reduced the hazard of liver transplant or liver-related death in patients who, after 1 year of UDCA monotherapy, had normal serum bilirubin (adjusted hazard ratio 0.09, 95% confidence interval 0.01-0.60, P = 0.013). Conclusion: Addition of BF to UDCA monotherapy improves not only GLOBE and UK-PBC scores but also the long-term prognosis of PBC patients, especially those with early-stage PBC.© 2019 by the American Association for the Study of Liver Diseases.

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