Article
Gastroenterology & Hepatology
Martine Walmsley, David Tornai, Nora Cazzagon, Angela Leburgue, Anna Mrzljak, Henrike Lenzen, Marco Carbone, Joao Madaleno, Ana Lleo, Norman Junge, Christoph Schramm, Annika Bergquist
Summary: The aim of this study was to assess patient-reported quality of care in primary sclerosing cholangitis (PSC) and identify areas for improvement. The results indicated that PSC patients experience a high symptom burden and would benefit from more widespread use of elastography, bone density scan, and appropriate treatment for itch. Personalized prognostic information and guidance on improving health should be provided to individuals with PSC.
LIVER INTERNATIONAL
(2023)
Article
Gastroenterology & Hepatology
Shekhar Singh Jadaun, Rohit Mehtani, Ana Hasnain, Sushant Bhatia, Vikash Moond, Mukesh Kumar, Vikash Kuhad, Shweta Singh, Shaleen Agarwal, Subhash Gupta, Sanjiv Saigal
Summary: This retrospective study analyzed the outcomes of living donor liver transplant (LDLT) in primary sclerosing cholangitis (PSC) at a tertiary liver transplant center in north India. The study found that LDLT in PSC can achieve good long-term outcomes, but there is a risk of PSC recurrence in approximately one-fifth of patients.
HEPATOLOGY INTERNATIONAL
(2023)
Review
Gastroenterology & Hepatology
Annarosa Floreani, Sara De Martin
Summary: PSC is a chronic cholestatic liver disease with no effective treatment currently available. There is controversy over the use of UDCA in PSC treatment. Novel pharmacological strategies targeting bile composition, immunomodulation, gut microbiome, and fibrosis are emerging, suggesting potential for personalized combination therapies for successful PSC treatment.
DIGESTIVE AND LIVER DISEASE
(2021)
Article
Gastroenterology & Hepatology
Zobair M. Younossi, Maria Stepanova, Issah Younossi, Andrei Racila
Summary: To fully understand the impact of PSC on patients' health, it is important to assess their HRQL. This study developed and validated a PSC-specific HRQL instrument using the CLDQ. The CLDQ-PSC showed good psychometric characteristics and can be used for patients with PSC.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Microbiology
Florian Fronhoffs, Leona Dold, Marijo Parcina, Arne Schneidewind, Maria Willis, Thomas F. E. Barth, Tobias J. Weismueller, Taotao Zhou, Philipp Lutz, Julian A. Luetkens, Peter Gerlach, Steffen Manekeller, Joerg C. Kalff, Tim O. Vilz, Christian P. Strassburg, Glen Kristiansen
Summary: Primary sclerosing cholangitis is a significant cause of liver transplantation. Hepatic alveolar echinococcosis, caused by Echinococcus multilocularis, can be detected by ultrasound or CT scan of the liver showing characteristic calcified conglomerates. Symptoms of alveolar echinococcosis occur after a long period of infection, accompanied by cholestasis or cholangitis. This report presents a case of presumed autoimmune hepatitis and primary sclerosing cholangitis in a patient, and post-transplantation diagnosis revealed alveolar echinococcosis in the explanted liver.
Article
Cell Biology
Nelli Sjoblom, Sonja Boyd, Hannu Kautiainen, Johanna Arola, Martti Farkkila
Summary: This study aimed to develop a histological classification system, the PSC histoscore, for PSC. The results showed that the PSC histoscore better correlated with clinical endpoints, and an elevated total PSC histoscore increased the risk for disease progression.
Article
Immunology
Emanual Maverakis, Alexander A. Merleev, Dayoung Park, Muchena J. Kailemia, Gege Xu, L. Renee Ruhaak, Kyoungmi Kim, Qiuting Hong, Qiongyu Li, Patrick Leung, William Liakos, Yu-Jui Yvonne Wan, Christopher L. Bowlus, Alina I. Marusina, Nelvish N. Lal, Yixuan Xie, Guillaume Luxardi, Carlito B. Lebrilla
Summary: The study utilized MRM mass spectrometry to characterize glycan alterations associated with PBC and PSC, resulting in multi-analyte diagnostic models capable of accurately distinguishing the diseases. Additionally, the research investigated how environmental factors, such as bile acids, can influence glycosylation and potentially lead to the generation of altered selfantigens as immune targets.
CLINICAL IMMUNOLOGY
(2021)
Article
Gastroenterology & Hepatology
Kim N. van Munster, Bregje Mol, Jorn C. Goet, Sanne N. van Munster, Rinse K. Weersma, Annemarie C. de Vries, Adriaan J. van der Meer, Akin Inderson, Joost P. Drenth, Karel J. van Erpecum, Kirsten Boonstra, Ulrich Beuers, Marcel G. W. Dijkgraaf, Cyriel Y. Ponsioen
Summary: This study aimed to assess the long-term burden of primary sclerosing cholangitis (PSC) in terms of survival, clinical course, quality adjusted life years (QALYs), medical consumption, and work productivity loss. The findings revealed substantial impact of PSC on patients' lives, with decreased life expectancy, impaired quality of life, increased medical costs, and work productivity loss.
LIVER INTERNATIONAL
(2023)
Article
Gastroenterology & Hepatology
Nora Cazzagon, Samantha Sarcognato, Annarosa Floreani, Giorgia Corra, Sara De Martin, Vincenza Guzzardo, Francesco Paolo Russo, Maria Guido
Summary: The study found that cellular senescence marker expression exists in cholangiocytes of PSC patients, which is related to the severity of the disease and patient outcomes. CS may serve as a new prognostic tool and potential therapeutic target in PSC.
Article
Gastroenterology & Hepatology
Michael Praktiknjo, Taotao Zhou, Maximiliane Krusken, Torid Jacob, Alois M. Sprinkart, Sebastian Nowak, Markus Kimmann, Leona Dold, Johannes Chang, Christian Jansen, Christian P. Strassburg, Julian Luetkens, Tobias J. Weismuller
Summary: This study found that intramuscular fat fraction (IMFF) is associated with transplant-free survival in patients with primary sclerosing cholangitis (PSC). IMFF may serve as an indicator for identifying patients in urgent need of liver transplantation.
DIGESTIVE AND LIVER DISEASE
(2023)
Article
Medicine, Research & Experimental
Shouyan Wu, Yuhan Cao, Henglei Lu, Xinming Qi, Jianhua Sun, Yang Ye, Likun Gong
Summary: This study identified the upregulation of chemokines and fibrosis in PSC patients and the significant ductular reaction and PBG proliferation in the chronic PSC model. In vitro experiments showed that MCP-1 secreted by cholangiocytes in PBG niche recruited macrophages and promoted inflammation and fibrosis. Further research revealed that POU6F1 could activate MCP-1 transcription, and 18 beta-Glycyrrhetinic acid (GA) reduced macrophages and fibrosis, benefiting acute and chronic PSC models. These findings provide potential targets and a theoretical basis for the clinical treatment of PSC.
BIOMEDICINE & PHARMACOTHERAPY
(2022)
Article
Gastroenterology & Hepatology
Qiaoyan Liu, Bo Li, Yikang Li, Yiran Wei, Bingyuan Huang, Jubo Liang, Zhengrui You, You Li, Qiwei Qian, Rui Wang, Jun Zhang, Ruiling Chen, Zhuwan Lyu, Yong Chen, Mingxia Shi, Xiao Xiao, Qixia Wang, Qi Miao, Jing-Yuan Fang, Merrill Eric Gershwin, Min Lian, Xiong Ma, Ruqi Tang
Summary: Comparative analysis of gut microbiome and metabolome in IgG4-SC and PSC patients revealed distinct host-microbe interactions that may contribute to disease pathogenesis, highlighting the uniqueness of IgG4-SC.
Article
Surgery
Wisam Sbeit, Tawfik Khoury, Eran Goldin, Mahmud Mahamid
Summary: The study found that fully-covered metal stent (FCMS) was a safe and effective treatment option for patients with Primary Sclerosing Cholangitis (PSC) related refractory Dominant Strictures (DS).
SURGICAL ENDOSCOPY AND OTHER INTERVENTIONAL TECHNIQUES
(2022)
Article
Gastroenterology & Hepatology
Jessica T. Hochberg, Aalam Sohal, Priya Handa, Bryan D. Maliken, Take-Kyun Kim, Kai Wang, Eric Gochanour, Yu Li, J. Bart Rose, James E. Nelson, Keith D. Lindor, Nicholas F. LaRusso, Kris V. Kowdley
Summary: This study investigated the changes in serum miRNA and cytokine profiles in patients with PSC treated with high-dose ursodeoxycholic acid (hd-UDCA) or placebo. The results showed significant alterations in serum miRNA profiles after hd-UDCA treatment, suggesting a potential link to liver toxicity.
Article
Rheumatology
Susan Bridgewater, Mwidimi Ndosi, Jill Dawson, Pamela Richards, Christine Silverthorne, Emma Dures, Susan M. Goodman, Catherine Hill, Sarah L. Mackie, Joanna C. Robson
Summary: The Steroid PRO is a 15-item scale that has been found to be valid and reliable for measuring the impact of glucocorticoid therapy in people with rheumatic diseases. The study included participants from the UK, USA, Australia and New Zealand, with a total of 946 responses. The scale structure was determined through Rasch measurement model and exploratory factor analysis, confirming its construct validity and reliability.
ANNALS OF THE RHEUMATIC DISEASES
(2023)
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
Cynthia Levy, Stuart Kendrick, Christopher L. Bowlus, Atsushi Tanaka, David Jones, Andreas E. Kremer, Marlyn J. Mayo, Nazneen Haque, Robyn von Maltzahn, Matthew Allinder, Brandon Swift, Megan M. McLaughlin, Gideon M. Hirschfield, GLIMMER Study Grp
Summary: GLIMMER evaluated the dose-response, efficacy, and safety of linerixibat in the treatment of cholestatic pruritus associated with primary biliary cholangitis (PBC). The study found that linerixibat did not show significant difference in itch reduction compared to placebo in the primary intent-to-treat analysis, but it demonstrated a significant dose-dependent reduction in itch in the per protocol population. A well-tolerated dose was identified for Phase 3 investigation in the treatment of cholestatic pruritus in PBC.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2023)
Letter
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)
Editorial Material
Gastroenterology & Hepatology
Christopher L. Bowlus, Cynthia Levy
Article
Oncology
Al B. Benson, Michael I. D'Angelica, Thomas Abrams, Daniel E. Abbott, Aijaz Ahmed, Daniel A. Anaya, Robert Anders, Chandrakanth Are, Melinda Bachini, David Binder, Mitesh Borad, Christopher Bowlus, Daniel Brown, Adam Burgoyne, Jason Castellanos, Prabhleen Chahal, Jordan Cloyd, Anne M. Covey, Evan S. Glazer, William G. Hawkins, Renuka Iyer, Rojymon Jacob, Lawrence Jennings, R. Kate Kelley, Robin Kim, Matthew Levine, Manisha Palta, James O. Park, Steven Raman, Sanjay Reddy, Sean Ronnekleiv-Kelly, Vaibhav Sahai, Gagandeep Singh, Stacey Stein, Anita Turk, Jean-Nicolas Vauthey, Alan P. Venook, Adam Yopp, Nicole McMillian, Ryan Schonfeld, Cindy Hochstetler
Summary: In 2023, the NCCN Guidelines for Hepatobiliary Cancers were divided into two separate guidelines: Hepatocellular Carcinoma and Biliary Tract Cancers. The Biliary Tract Cancers guidelines provide recommendations for the evaluation and comprehensive care of patients with gallbladder cancer, intrahepatic cholangiocarcinoma, and extrahepatic cholangiocarcinoma. A multidisciplinary panel of experts meets regularly to review new data and therapies.
JOURNAL OF THE NATIONAL COMPREHENSIVE CANCER NETWORK
(2023)
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)