Article
Gastroenterology & Hepatology
Christophe Corpechot, Fabrice Carrat, Farid Gaouar, Frederic Chau, Gideon Hirschfield, Aliya Gulamhusein, Aldo J. Montano-Loza, Ellina Lytvyak, Christoph Schramm, Albert Pares, Ignasi Olivas, John E. Eaton, Karim T. Osman, George Dalekos, Nikolaos Gatselis, Frederik Nevens, Nora Cazzagon, Alessandra Zago, Francesco Paolo Russo, Nadir Abbas, Palak Trivedi, Douglas Thorburn, Francesca Saffioti, Laszlo Barkai, Davide Roccarina, Vicenza Calvaruso, Anna Fichera, Adele Delamarre, Esli Medina-Morales, Alan Bonder, Vilas Patwardhan, Cristina Rigamonti, Marco Carbone, Pietro Invernizzi, Laura Cristoferi, Adriaan van der Meer, Rozanne de Veer, Ehud Zigmond, Eyal Yehezkel, Andreas E. Kremer, Ansgar Deibel, Jerome Dumortier, Tony Bruns, Karsten Grosse, Georges-Philippe Pageaux, Aaron Wetten, Jessica Dyson, David Jones, Olivier Chazouilleres, Bettina Hansen, Victor de Ledinghen
Summary: In the largest ever study of LSM in patients with PBC, LSM assessed by VCTE improves prediction of survival beyond biochemical response, established prognostic scores, and age categories, independent of time. LSM can reasonably be regarded as a useful prognostic marker and a potential surrogate endpoint in PBC clinical trials.
JOURNAL OF HEPATOLOGY
(2022)
Article
Gastroenterology & Hepatology
Eric F. Martin
Summary: The number of liver transplantation for primary biliary cholangitis (PBC) has decreased over the years, but it remains an important and curative option for progressive PBC patients. Recent studies from the Global PBC Study Group have provided valuable insights into the changes in PBC before and after liver transplantation.
CLINICS IN LIVER DISEASE
(2022)
Article
Gastroenterology & Hepatology
Cynthia Levy, Michael Manns, Gideon Hirschfield
Summary: Primary biliary cholangitis is an autoimmune disease with symptoms including fatigue, itch, abdominal pain, and sicca complex. Treatment focuses on cholestatic consequences.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Review
Pharmacology & Pharmacy
Aaron Wetten, David Emrys Jeffreys Jones, Jessica Katharine Dyson
Summary: This article summarizes the use of generic fibrates as add-in therapy for PBC and evaluates the clinical trial data of seladelpar in terms of liver biochemistry, symptomology, and safety. Results show that seladelpar leads to marked improvement in liver biochemistry and symptoms, and safety concerns have been addressed. Compared to existing off-label fibrates, seladelpar has the potential to be an attractive second-line agent in PBC.
EXPERT OPINION ON INVESTIGATIONAL DRUGS
(2022)
Article
Gastroenterology & Hepatology
Ellina Lytvyak, Mina Niazi, Rohit Pai, Daniel He, Guangzhi Zhang, Stefan G. Hubscher, Andrew L. Mason
Summary: Managment of recurrent primary biliary cholangitis with combination antiretroviral therapy has shown successful outcomes, although it can be associated with side effects.
LIVER INTERNATIONAL
(2021)
Article
Gastroenterology & Hepatology
Jorn C. Goet, Carla F. Murillo Perez, Maren H. Harms, Annarosa Floreani, Nora Cazzagon, Tony Bruns, Florian Prechter, George N. Dalekos, Xavier Verhelst, Nikolaos K. Gatselis, Keith D. Lindor, Willem J. Lammers, Aliya Gulamhusein, Anna Reig, Marco Carbone, Frederik Nevens, Gideon M. Hirschfield, Adriaan J. van der Meer, Henk R. van Buuren, Bettina E. Hansen, Albert Pares
Summary: This study evaluated the prognostic value of Mayo Risk Score (MRS), UK-PBC score, and GLOBE score in patients with primary biliary cholangitis, finding that the GLOBE score had superior discriminatory performance. However, all scores demonstrated comparable prediction accuracy for outcomes such as liver transplantation or death.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2021)
Article
Gastroenterology & Hepatology
Mark R. Pedersen, Garrett Greenan, Sumant Arora, Arvind R. Murali, Marlyn J. Mayo
Summary: UDCA after LT reduces the odds of BC and bile stones and sludge in all-comer LT recipients and reduces or delays the incidence of rPBC in patients transplanted for PBC. UDCA use after LT could be considered in all LT recipients to reduce the odds of BC and may be particularly beneficial for patients transplanted for PBC by reducing the incidence of rPBC.
LIVER TRANSPLANTATION
(2021)
Article
Pathology
Maylee Hsu, Jennifer Y. Ju, Meredith M. Pearson, Lei Yu, Paul E. Swanson, Matthew M. Yeh
Summary: This study found that the number of IgM-positive plasma cells is higher in explants of cirrhotic PBC compared with AIH, which may be helpful in the evaluation of cryptogenic cirrhosis.
AMERICAN JOURNAL OF CLINICAL PATHOLOGY
(2022)
Article
Gastroenterology & Hepatology
Atsushi Tanaka, Junko Hirohara, Toshiaki Nakano, Kosuke Matsumoto, Olivier Chazouilleres, Hajime Takikawa, Bettina E. Hansen, Fabrice Carrat, Christophe Corpechot
Summary: The addition of bezafibrate to ursodeoxycholic acid in patients with primary biliary cholangitis was associated with improved prognosis, as evident from a large retrospective cohort study. The combination therapy showed a significant decrease in all-cause and liver-related mortality or need for liver transplantation, indicating the potential benefits of this treatment approach.
JOURNAL OF HEPATOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Yukihisa Fujinaga, Tadashi Namisaki, Yuki Tsuji, Junya Suzuki, Koji Murata, Soichi Takeda, Hiroaki Takaya, Takashi Inoue, Ryuichi Noguchi, Yuki Fujimoto, Masahide Enomoto, Norihisa Nishimura, Koh Kitagawa, Kosuke Kaji, Hideto Kawaratani, Takemi Akahane, Akira Mitoro, Hitoshi Yoshiji
Summary: Primary biliary cholangitis (PBC) presents with a wide variation in clinical symptoms and course. This study investigated surrogate markers for risk estimation of PBC-related complications and found that serum soluble mannose receptor (sMR) can predict the development of complications in PBC patients. The combination of sMR and soluble CD163 (sCD163) shows better predictive power than sCD163 alone, but the addition of sCD163 does not improve the predictive ability of sMR. These findings contribute to the development of biomarkers for identifying high-risk PBC patients.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Pharmacology & Pharmacy
Chenyi Xu, Rensong Yue, Xuelian Lv, Shengnan Wang, Mengmeng Du
Summary: This systematic review and meta-analysis evaluated the efficacy and safety of drug interventions in the treatment of pruritus associated with primary biliary cholangitis. The results showed that UDCA, methotrexate, and GSK2330672 can relieve itching, but there is heterogeneity in their effects on serum indexes, and no specific drug can be recommended for the treatment of pruritus based on the current evidence.
FRONTIERS IN PHARMACOLOGY
(2022)
Article
Gastroenterology & Hepatology
Raj Vuppalanchi, Stephen H. Caldwell, Nikolaos Pyrsopoulos, Andrew S. DeLemos, Simona Rossi, Cynthia Levy, David S. Goldberg, Edward A. Mena, Aasim Sheikh, Ravi Ravinuthala, Farheen Shaikh, James D. Bainbridge, Deven Parmar, Naga P. Chalasani
Summary: The study showed that daily dosages of 2 mg and 4 mg saroglitazar were well tolerated and resulted in rapid and sustained improvements in ALP levels in patients with PBC. It was noted that the 4 mg dose led to a higher incidence of elevated liver enzymes.
JOURNAL OF HEPATOLOGY
(2022)
Review
Immunology
Yushu Yang, XiaoSong He, Manuel Rojas, Patrick S. C. Leung, Lixia Gao
Summary: Primary biliary cholangitis (PBC) is an immune-mediated liver disease characterized by cholestasis, biliary injuries, liver fibrosis, and chronic non-suppurative cholangitis. The pathogenesis of PBC involves immune dysregulation, abnormal bile metabolism, and progressive fibrosis, leading to cirrhosis and liver failure. Current treatments have limitations, and there is a need for further research to investigate underlying mechanisms and develop more effective therapies.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Health Care Sciences & Services
Alessio Gerussi, Damiano Verda, Claudio Cappadona, Laura Cristoferi, Davide Paolo Bernasconi, Sandro Bottaro, Marco Carbone, Marco Muselli, Pietro Invernizzi, Rosanna Asselta
Summary: This study evaluated the feasibility and accuracy of using a machine learning model for disease risk prediction in Primary Biliary Cholangitis (PBC). The results showed that the model had high accuracy and specificity, and can be used for disease prediction in individuals at risk.
JOURNAL OF PERSONALIZED MEDICINE
(2022)
Review
Gastroenterology & Hepatology
Helen T. Smith, Andrea Ribeiro de Souza, April H. Thompson, Megan M. McLaughlin, John J. Dever, Julie A. Myers, Jing Voon Chen
Summary: This study conducted a systematic literature review to investigate the evidence supporting treatment decisions for cholestatic pruritus associated with PBC and PSC. The findings showed a lack of consistent and reproducible evidence on the efficacy, impact on HRQoL, and safety of cholestatic pruritus treatments, leading physicians to rely on clinical experience for treatment selection.
DIGESTIVE DISEASES AND SCIENCES
(2023)