Article
Radiology, Nuclear Medicine & Medical Imaging
Yun Zhang, Xiaoli Fan, Bin Song, Yifeng Liu, Yidi Chen, Tianying Zheng, Yuxin Guo, Ting Duan, Zixing Huang, Li Yang
Summary: This study explored the feasibility of using nonenhanced magnetic resonance imaging (MRI) to predict insufficient biochemical response to ursodeoxycholic acid (UDCA) in patients with primary biliary cholangitis (PBC). The results showed that a predictive model based on three pretreatment MRI features could accurately predict insufficient biochemical response to UDCA treatment in PBC patients within 12 months. Early identification of high-risk patients can facilitate the timely initiation of additional treatment.
EUROPEAN RADIOLOGY
(2023)
Article
Gastroenterology & Hepatology
Kazuhito Kawata, Satoru Joshita, Shinji Shimoda, Yuki Yamashita, Maho Yamashita, Kensuke Kitsugi, Shingo Takatori, Kazuyoshi Ohta, Jun Ito, Shin Shimoyama, Hidenao Noritake, Takafumi Suda, Kenichi Harada
Summary: The URS can predict the biochemical response to UDCA treatment in PBC patients, as well as reflect histopathological features before treatment. This scoring system can identify inadequate histological response to UDCA treatment in the Scheuer classification and the Nakanuma system.
HEPATOLOGY RESEARCH
(2021)
Article
Pharmacology & Pharmacy
Chunlei Li, Kunyu Zheng, Yiran Chen, Chengmei He, Suying Liu, Yunjiao Yang, Mengtao Li, Xiaofeng Zeng, Li Wang, Fengchun Zhang
Summary: This study examined the efficacy and safety of combining fenofibrate with ursodeoxycholic acid (UDCA) in the treatment of PBC patients. The results showed that the addition of fenofibrate can improve biochemical indexes of PBC patients who had an incomplete response to UDCA.
THERAPEUTIC ADVANCES IN CHRONIC DISEASE
(2022)
Article
Gastroenterology & Hepatology
Lin-Xiang Huang, Zi-Long Wang, Rui Jin, Hong-Song Chen, Bo Feng
Summary: Primary biliary cholangitis (PBC) is a common autoimmune disease that leads to increased mortality among end-stage liver disease (ESLD) patients waiting for liver transplantation. Ursodeoxycholic acid (UDCA) is the only approved first-line medicine for PBC and a good response to treatment can improve prognosis.
EXPERT REVIEW OF GASTROENTEROLOGY & HEPATOLOGY
(2022)
Article
Gastroenterology & Hepatology
Anna Reig, Carmen Alvarez-Navascues, Mercedes Vergara, Elena Gomez-Dominguez, Adolfo Gallego-Moya, Indhira M. Perez-Medrano, Emilio Fabrega, Manuel Hernandez-Guerra, Marina Berenguer, Pamela Estevez, Ana Arencibia, Rosa M. Morillas, Diana Horta, Agustin Albillos, Marta Casado, Gema De la Cruz, Eva Fernandez-Bonilla, Esther Molina, Lander Hijona, Moises Diago, Conrado M. Fernandez-Rodriguez, Jesus M. Gonzalez-Santiago, Margarita Sala, Judith Gomez-Camarero, Manuel Romero-Gomez, Francisco Suarez, Victor Vargas, Carlos Ferre-Aracil, Raul J. Andrade, Nadia Chahri, Albert Pares
Summary: The therapy of OCA and fibrates can improve hepatic biochemistry and the GLOBE score in patients with primary biliary cholangitis, and simultaneous treatment with both can also improve ALP levels.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2021)
Review
Gastroenterology & Hepatology
Tadashi Namisaki, Yukihisa Fujinaga, Kei Moriya, Hitoshi Yoshiji
Summary: PBC patients with suboptimal response to UDCA are at high risk for end-stage liver disease, requiring new surrogate endpoints to identify candidates for second-line therapies, as current staging systems are insufficient in determining disease severity and progression.
HEPATOLOGY RESEARCH
(2021)
Review
Biochemistry & Molecular Biology
Annarosa Floreani, Daniela Gabbia, Sara De Martin
Summary: Primary biliary cholangitis (PBC) is a rare autoimmune liver disease with limited treatment options. Ursodeoxycholic acid (UDCA) is the first-line therapy but is ineffective for a significant portion of patients. Obeticholic acid (OCA) is an effective second-line treatment for UDCA-non responders. Future therapies for PBC are promising.
Article
Gastroenterology & Hepatology
Helena Cortez-Pinto, Rodrigo Liberal, Susana Lopes, Mariana Machado, Joana Carvalho, Teresa Dias, Arsenio Santos, Claudia Agostinho, Pedro Figueiredo, Rafaela Loureiro, Alexandra Martins, Goncalo Alexandrino, Isabel Cotrim, Carina Leal, Jose Presa, Monica Mesquita, Joana Nunes, Catarina Gouveia, Ana Horta E. Vale, Ana Luisa Alves, Mariana Coelho, Luis Maia, Isabel Pedroto, Antonio Banhudo, Joao Sebastiao Pinto, Marta Vargas Gomes, Joana Oliveira, Valeska Andreozzi, Filipe Calinas
Summary: This study found a considerable proportion of patients with primary biliary cholangitis showed incomplete biochemical response to ursodeoxycholic acid (UDCA) treatment. Patients with cirrhosis, elevated levels of gamma-glutamyl transferase (GGT) and alkaline phosphatase (ALP) at diagnosis were identified as being at higher risk for incomplete response. Early identification of patients at risk of incomplete response could improve treatment care and guide clinical decision-making.
UNITED EUROPEAN GASTROENTEROLOGY JOURNAL
(2021)
Review
Gastroenterology & Hepatology
Kris. V. V. Kowdley, Christopher. L. L. Bowlus, Cynthia Levy, Marlyn. J. J. Mayo, Daniel. S. S. Pratt, Raj Vuppalanchi, Zobair. M. M. Younossi
Summary: Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune liver disease. There have been recent important developments in the diagnosis, treatment, and monitoring of patients with PBC. In this updated expert consensus document, we provide updates on staging, noninvasive prognostic tools, and a treatment algorithm for clinicians managing PBC.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2023)
Article
Gastroenterology & Hepatology
Dawei Ding, Guoyun Xuan, Yinan Hu, Jiahao Yu, Yansheng Liu, Guanya Guo, Shuoyi Ma, Fangfang Yang, Siyuan Tian, Gang Ma, Ling Chen, Xinmin Zhou, Yulong Shang, Ying Han
Summary: For patients with seropositive anti-mitochondrial antibody but normal alkaline phosphatase, a liver biopsy may be necessary to diagnose primary biliary cholangitis (PBC). This study found that higher baseline serum immunoglobulin M levels and older age were associated with a diagnosis of PBC based on liver biopsies. For patients receiving ursodeoxycholic acid treatment, monitoring of gamma-glutamyl transpeptidase and immunoglobulin M levels may be helpful in evaluating therapeutic responses.
HEPATOLOGY COMMUNICATIONS
(2022)
Article
Gastroenterology & Hepatology
Omar Y. Mousa, Brian D. Juran, Bryan M. McCauley, Mette N. Vesterhus, Trine Folseraas, Coleman T. Turgeon, Ahmad H. Ali, Erik M. Schlicht, Elizabeth J. Atkinson, Chang Hu, Denise Harnois, Elizabeth J. Carey, Andrea A. Gossard, Devin Oglesbee, John E. Eaton, Nicholas F. LaRusso, Gregory J. Gores, Tom H. Karlsen, Konstantinos N. Lazaridis
Summary: In patients with PSC, alterations in plasma BA consistent with cholestasis, UDCA treatment, and IBD were observed. BA profiles were able to predict future hepatic decompensation, demonstrating the clinical potential of BA profiling for use in clinical trials.
Review
Biochemistry & Molecular Biology
Annarosa Floreani, Daniela Gabbia, Sara De Martin
Summary: Ursodeoxycholic acid is the first-line therapy for PBC, but new pharmacological agents are being proposed for UDCA-non-responders. Obeticholic acid and fibrates have shown effectiveness in improving biochemistry alteration and symptoms of PBC. Other new agents with different mechanisms of action are also under clinical evaluation for PBC treatment.
Article
Immunology
Haolong Li, Haoting Zhan, Linlin Cheng, Yuan Huang, Xiaomeng Li, Songxin Yan, Yongmei Liu, Li Wang, Yongzhe Li
Summary: This study analyzed the plasma lipid profiles of patients with primary biliary cholangitis (PBC) and Sjogren's syndrome (SS). The results showed that PBC patients had 115 upregulated lipids compared to healthy controls, and 17 lipids were associated with disease activity and could differentiate UDCA responders from non-responders. Compared to SS, PBC patients had 44 upregulated lipids, and 8 lipids were identified as potential diagnostic markers for PBC.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Gastroenterology & Hepatology
Yuki Yamashita, Takeji Umemura, Takefumi Kimura, Satoru Joshita, Junko Hirohara, Toshiaki Nakano, Atsumasa Komori, Atsushi Tanaka
Summary: This study showed that baseline ALBI grade is a simple non-invasive predictor for prognosis in PBC, and it is significantly associated with histological stage and disease progression. ALBI score/grade can be used to assess liver function and predict mortality and the need for liver transplantation in PBC patients.
Article
Gastroenterology & Hepatology
Gideon M. Hirschfield, Mitchell L. Shiffman, Aliya Gulamhusein, Kris Kowdley, John M. Vierling, Cynthia Levy, Andreas E. Kremer, Ehud Zigmond, Pietro Andreone, Stuart C. Gordon, Christopher L. Bowlus, Eric J. Lawitz, Richard J. Aspinall, Daniel S. Pratt, Karina Raikhelson, Maria S. Gonzalez-Huezo, Michael A. Heneghan, Sook-Hyang Jeong, Alma L. Ladron de Guevara, Marlyn J. Mayo, George N. Dalekos, Joost P. H. Drenth, Ewa Janczewska, Barbara A. Leggett, Frederik Nevens, Victor Vargas, Eli Zuckerman, Christophe Corpechot, Eduardo Fassio, Holger Hinrichsen, Pietro Invernizzi, Palak J. Trivedi, Lisa Forman, David E. J. Jones, Stephen D. Ryder, Mark G. Swain, Alexandra Steinberg, Pol F. Boudes, Yun-Jung Choi, Charles A. McWherter
Summary: This study evaluated the efficacy and safety of seladelpar in patients with primary biliary cholangitis who had inadequate response or intolerance to ursodeoxycholic acid. The results showed that seladelpar significantly improved liver biochemistry and pruritus in these patients, and it was well tolerated.
Article
Gastroenterology & Hepatology
Michael Trauner, Christopher L. Bowlus, Aliya Gulamhusein, Bilal Hameed, Stephen H. Caldwell, Mitchell L. Shiffman, Charles Landis, Andrew J. Muir, Andrew Billin, Jun Xu, Xiangyu Liu, Xiaomin Lu, Chuhan Chung, Robert P. Myers, Kris V. Kowdley
Summary: In this 96-week open-label extension study, Cilofexor was found to be safe and effective in improving liver function and biomarkers of cholestasis and cellular injury in patients with PSC.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Article
Gastroenterology & Hepatology
Marlyn J. Mayo, Elizabeth Carey, Helen T. Smith, Andrea R. Mospan, Megan McLaughlin, April Thompson, Heather L. Morris, Robert Sandefur, W. Ray Kim, Christopher Bowlus, Cynthia Levy
Summary: This study investigated the impact of pruritus on quality of life and its management in patients with PBC. The prevalence of pruritus was found to be high and it had a negative impact on patients' quality of life. However, the study also found that pruritus in PBC is often undertreated.
DIGESTIVE DISEASES AND SCIENCES
(2023)
Article
Gastroenterology & Hepatology
Jun Xu, Ya Wang, Mina Khoshdeli, Matt Peach, Jen-Chieh Chuang, Julie Lin, Wen-Wei Tsai, Sangeetha Mahadevan, Wesley Minto, Lauri Diehl, Ruchi Gupta, Michael Trauner, Keyur Patel, Mazen Noureddin, Kris Kowdley, Aliya Gulamhusein, Christopher L. Bowlus, Ryan S. Huss, Robert P. Myers, Chuhan Chung, Andrew N. Billin
Summary: This study evaluated IL-31 as a potential biomarker for pruritus in clinical trials of cilofexor, an FXR agonist, in patients with cholestatic liver diseases. IL-31 levels were found to be elevated in patients with PSC and PBC and correlated with serum bile acids. The study also showed that cilofexor therapy increased IL-31 levels in patients with NASH.
Article
Gastroenterology & Hepatology
Na Sun, Ceng Zhang, Yi-Te Lee, Benjamin V. Tran, Jing Wang, Hyoyong Kim, Junseok Lee, Ryan Y. Zhang, Jasmine J. Wang, Junhui Hu, Zhicheng Zhang, Manaf S. Alsudaney, Kuan-Chu Hou, Hubert Tang, Tiffany X. Zhang, Icy Y. Liang, Ziang Zhou, Mengxiang Chen, Angela Hsiao-Jiun Yeh, Wenyuan Li, Xianghong Jasmine Zhou, Helena R. Chang, Steven-Huy B. Han, Saeed Sadeghi, Richard S. Finn, Sammy Saab, Ronald W. Busuttil, Mazen Noureddin, Walid S. Ayoub, Alexander Kuo, Vinay Sundaram, Buraq Al-Ghaieb, Juvelyn Palomique, Kambiz Kosari, Irene K. Kim, Tsuyoshi Todo, Nicholas N. Nissen, Maria Lauda Tomasi, Sungyong You, Edwin M. Posadas, James X. Wu, Madhuri Wadehra, Myung-Shin Sim, Yunfeng Li, Hanlin L. Wang, Samuel W. French, Shelly C. Lu, Lily Wu, Renjun Pei, Li Liang, Ju Dong Yang, Vatche G. Agopian, Hsian-Rong Tseng, Yazhen Zhu
Summary: The study developed an HCC EV-based surface protein assay for early detection of HCC, showing promising potential in detecting early-stage HCC.
Article
Gastroenterology & Hepatology
Christopher L. Bowlus, Lionel Arrive, Annika Bergquist, Mark Deneau, Lisa Forman, Sumera I. Ilyas, Keri E. Lunsford, Mercedes Martinez, Gonzalo Sapisochin, Rachna Shroff, James H. Tabibian, David N. Assis
Letter
Gastroenterology & Hepatology
Lisa M. M. Forman, Gonzalo Sapisochin, David N. N. Assis, Lionel Arrive, Annika Bergquist, Christopher L. L. Bowlus, Mark Deneau, Sumera I. I. Ilyas, Keri E. E. Lunsford, Mercedes Martinez, Rachna Shroff, James H. H. Tabibian
Article
Gastroenterology & Hepatology
Eric W. W. Chak, Randy Luna, Scott MacDonald, Susan L. L. Stewart, Moon S. S. Chen Jr, Christopher Bowlus
JOURNAL OF VIRAL HEPATITIS
(2023)
Article
Gastroenterology & Hepatology
Michael Trauner, Chuhan Chung, Kate Sterling, Xiangyu Liu, Xiaomin Lu, Jun Xu, Clare Tempany-Afdhal, Zachary D. Goodman, Martti Faerkkilae, Atsushi Tanaka, Palak Trivedi, Kris V. Kowdley, Christopher L. Bowlus, Cynthia Levy, Robert P. Myers
Summary: This article presents the background of PSC and the design and implementation of the phase 3 PRIMIS trial, which aims to evaluate the efficacy and safety of cilofexor in noncirrhotic patients with large-duct PSC. The primary objective is to assess whether cilofexor reduces the risk of fibrosis progression compared with placebo.
BMC GASTROENTEROLOGY
(2023)
Article
Gastroenterology & Hepatology
Gideon M. Hirschfield, Mitchell L. Shiffman, Aliya Gulamhusein, Kris Kowdley, John M. Vierling, Cynthia Levy, Andreas E. Kremer, Ehud Zigmond, Pietro Andreone, Stuart C. Gordon, Christopher L. Bowlus, Eric J. Lawitz, Richard J. Aspinall, Daniel S. Pratt, Karina Raikhelson, Maria S. Gonzalez-Huezo, Michael A. Heneghan, Sook-Hyang Jeong, Alma L. Ladron de Guevara, Marlyn J. Mayo, George N. Dalekos, Joost P. H. Drenth, Ewa Janczewska, Barbara A. Leggett, Frederik Nevens, Victor Vargas, Eli Zuckerman, Christophe Corpechot, Eduardo Fassio, Holger Hinrichsen, Pietro Invernizzi, Palak J. Trivedi, Lisa Forman, David E. J. Jones, Stephen D. Ryder, Mark G. Swain, Alexandra Steinberg, Pol F. Boudes, Yun-Jung Choi, Charles A. McWherter
Summary: This study evaluated the efficacy and safety of seladelpar in patients with primary biliary cholangitis who had inadequate response or intolerance to ursodeoxycholic acid. The results showed that seladelpar significantly improved liver biochemistry and pruritus in these patients, and it was well tolerated.
Article
Gastroenterology & Hepatology
Christopher L. Bowlus, Bertus Eksteen, Angela C. Cheung, Douglas Thorburn, Cynthia A. Moylan, Paul J. Pockros, Lisa M. Forman, Alejandro Dorenbaum, Gideon M. Hirschfield, Ciara Kennedy, Thomas Jaecklin, Andrew McKibben, Elaine Chien, Marshall Baek, Pamela Vig, Cynthia Levy
Summary: Maralixibat, an IBAT inhibitor, was found to lower bile acid levels and improve itching symptoms in patients with PSC. The adverse events during treatment were mainly gastrointestinal-related and mostly mild or moderate. These results support further investigation of IBAT inhibitors for the treatment of PSC-associated pruritus.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
David N. Assis, Christopher L. Bowlus
Summary: Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by fibroinflammatory damage to the biliary tree. It is often associated with inflammatory bowel disease and carries an increased risk of liver failure and cholangiocarcinoma. The complexity of the disease and lack of effective therapy have been challenges, but recent advancements in patient characterization, prognostication, and medical management offer hope for progress in the future. This review summarizes current understanding of PSC and highlights areas for further investigation.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Correction
Gastroenterology & Hepatology
Marlyn Mayo, Elizabeth T. Carey, Helen R. Smith, Andrea Mospan, Megan McLaughlin, April L. Thompson, Heather Morris, Robert Sandefur, W. Ray Kim, Christopher Bowlus, TARGET PBC Investigators, Cynthia Levy
DIGESTIVE DISEASES AND SCIENCES
(2023)
Editorial Material
Gastroenterology & Hepatology
Christopher L. Bowlus, Cynthia Levy
Review
Gastroenterology & Hepatology
Kris. V. V. Kowdley, Christopher. L. L. Bowlus, Cynthia Levy, Marlyn. J. J. Mayo, Daniel. S. S. Pratt, Raj Vuppalanchi, Zobair. M. M. Younossi
Summary: Primary biliary cholangitis (PBC) is a chronic, cholestatic, autoimmune liver disease. There have been recent important developments in the diagnosis, treatment, and monitoring of patients with PBC. In this updated expert consensus document, we provide updates on staging, noninvasive prognostic tools, and a treatment algorithm for clinicians managing PBC.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2023)
Article
Radiology, Nuclear Medicine & Medical Imaging
Erick Cruz Grave, Thomas Loehfelm, Michael T. Corwin, Joseph Zepeda, Harjot K. Bath, Sandeep Dhaliwal, Maryam Yazdanfar, Christopher L. Bowlus
Summary: This study found that the Anali score provides additional predictive value for hepatic decompensation in patients with PSC. The Anali score has good discriminative ability with the Mayo risk score and can serve as an independent predictor.
ABDOMINAL RADIOLOGY
(2023)