It is time to change primary biliary cirrhosis (PBC): New nomenclature from "cirrhosis" to "cholangitis", and upcoming treatment based on unveiling pathology

Shinji Shimoda, Atsushi Tanaka

Research output: Contribution to journalReview article

3 Citations (Scopus)

Abstract

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.

Original languageEnglish
Pages (from-to)407-415
Number of pages9
JournalHepatology Research
Volume46
Issue number5
DOIs
Publication statusPublished - Mar 1 2016

Fingerprint

Cholangitis
Biliary Liver Cirrhosis
Terminology
Fibrosis
Ursodeoxycholic Acid
Pathology
Bezafibrate
Japan
Therapeutics
Genome-Wide Association Study
Cholestasis
Adaptive Immunity
Gastroenterology
Bile Ducts
Innate Immunity
Autoimmune Diseases
Names
Liver Diseases
Clinical Trials
Drug Therapy

All Science Journal Classification (ASJC) codes

  • Hepatology
  • Infectious Diseases

Cite this

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title = "It is time to change primary biliary cirrhosis (PBC): New nomenclature from {"}cirrhosis{"} to {"}cholangitis{"}, and upcoming treatment based on unveiling pathology",
abstract = "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.",
author = "Shinji Shimoda and Atsushi Tanaka",
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T1 - It is time to change primary biliary cirrhosis (PBC)

T2 - New nomenclature from "cirrhosis" to "cholangitis", and upcoming treatment based on unveiling pathology

AU - Shimoda, Shinji

AU - Tanaka, Atsushi

PY - 2016/3/1

Y1 - 2016/3/1

N2 - 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.

AB - 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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