Biochemical responses to bezafibrate improve long-term outcome in asymptomatic patients with primary biliary cirrhosis refractory to UDCA

Tanaka, Atsushi; Hirohara, Junko; Nakanuma, Yasuni; Tsubouchi, Hirohito; Takikawa, Hajime

doi:10.1007/s00535-014-0998-z

Cited by 37 publications

(42 citation statements)

References 33 publications

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“…Bezafibrate was more effective in non‐cirrhotic patients (liver stiffness value <14.4 kPa), albeit not significantly. Ursodeoxycholic acid is the drug used most frequently for PSC, and combination therapy using UDCA and bezafibrate is effective for primary biliary cholangitis . However, in this analysis, concomitant use of UDCA tended to be associated with bezafibrate inefficacy.…”

Section: Discussionmentioning

confidence: 63%

Factors predictive of the efficacy of bezafibrate therapy in patients with primary sclerosing cholangitis

Mizuno

Isayama

Hirano

et al. 2017

Hepatology Research

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Section: Discussionmentioning

confidence: 63%

Factors predictive of the efficacy of bezafibrate therapy in patients with primary sclerosing cholangitis

Mizuno

Isayama

Hirano

et al. 2017

Hepatology Research

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“…In contrast, when we calculated the HR of BF treatment after IPTW adjustment to reduce background selection bias, BF treatment significantly reduced the risk of liver transplantation or liver‐related death in patients with normal serum bilirubin. Furthermore, our previous long‐term (6.1 ± 3.4 years) observational study using 835 asymptomatic PBC patients from a Japanese nationwide survey showed that ALT response to BF treatment was significantly associated with the occurrence of liver‐related symptoms, which also suggests that the addition of BF to UDCA monotherapy improves the long‐term prognosis of PBC patients.…”

Section: Discussionmentioning

confidence: 87%

Bezafibrate Improves GLOBE and UK‐PBC Scores and Long‐Term Outcomes in Patients With Primary Biliary Cholangitis

et al. 2019

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In 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.

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“…Fibrates, including bezafibrate and fenofibrate, may be useful for treating asymptomatic patients with PBC who exhibit inappropriate response to UDCA[179-183]. A nationwide retrospective survey in Japan demonstrated that normalizing serum ALT concentrations with accessional bezafibrate therapy observably reduced the occurrence of hepatic illness-associated clinical signs in symptomless patients, with PBC responding incompletely to UDCA[179].…”

Section: Therapy Of Pbcmentioning

confidence: 99%

“…A nationwide retrospective survey in Japan demonstrated that normalizing serum ALT concentrations with accessional bezafibrate therapy observably reduced the occurrence of hepatic illness-associated clinical signs in symptomless patients, with PBC responding incompletely to UDCA[179]. Moreover, long-term combined bezafibrate and UDCA treatment in PBC not only observably ameliorated the Mayo risk score and serum concentrations of ALP, but also observably elevated the serum concentrations of creatinine[180].…”

Section: Therapy Of Pbcmentioning

confidence: 99%

Recent advances in the diagnosis and treatment of primary biliary cholangitis

Huang¹

2016

WJH

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Primary biliary cholangitis (PBC), formerly referred to as primary biliary cirrhosis, is an infrequent progressive intrahepatic cholestatic autoimmune illness that can evolve into hepatic fibrosis, hepatic cirrhosis, hepatic failure, and, in some cases, hepatocellular carcinoma. The disease itself is characterized by T-lymphocyte-mediated chronic non-suppurative destructive cholangitis and elevated serum levels of extremely specific anti-mitochondrial autoantibodies (AMAs). In this article, we will not only review epidemiology, risk factors, natural history, predictive scores, radiologic approaches (e.g., acoustic radiation force impulse imaging, vibration controlled transient elastography, and magnetic resonance elastography), clinical features, serological characteristics covering biochemical markers, immunoglobulins, infections markers, biomarkers, predictive fibrosis marker, specific antibodies (including AMAs such as AMA-M2), anti-nuclear autoantibodies [such as anti-multiple nuclear dot autoantibodies (anti-sp100, PML, NDP52, anti-sp140), anti-rim-like/membranous anti-nuclear autoantibodies (anti-gp210, anti-p62), anti-centromere autoantibodies, and some of the novel autoantibodies], histopathological characteristics of PBC, diagnostic advances, and anti-diastole of PBC. Furthermore, this review emphasizes the recent advances in research of PBC in terms of therapies, including ursodeoxycholic acid, budesonide, methotrexate, obeticholic acid, cyclosporine A, fibrates such as bezafibrate and fenofibrate, rituximab, mesenchymal stem cells transplant, and hepatic transplant. Currently, hepatic transplant remains the only optimal choice with acknowledged treatment efficiency for end-stage PBC patients.

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Biochemical responses to bezafibrate improve long-term outcome in asymptomatic patients with primary biliary cirrhosis refractory to UDCA

Abstract: These results suggested that normalization of ALT levels with additional bezafibrate treatment significantly decreased the rate of occurrence of liver-related symptoms in asymptomatic PBC patients with suboptimal response to UDCA.

Cited by 37 publications

References 33 publications

Factors predictive of the efficacy of bezafibrate therapy in patients with primary sclerosing cholangitis

Factors predictive of the efficacy of bezafibrate therapy in patients with primary sclerosing cholangitis

Bezafibrate Improves GLOBE and UK‐PBC Scores and Long‐Term Outcomes in Patients With Primary Biliary Cholangitis

Recent advances in the diagnosis and treatment of primary biliary cholangitis

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