Article
Gastroenterology & Hepatology
Kris V. Kowdley, Lisa Forman, Bertus Eksteen, Nadege Gunn, Vinay Sundaram, Charles Landis, Stephen A. Harrison, Cynthia Levy, Alexander Liberman, Adrian M. Di Bisceglie, Gideon M. Hirschfield
Summary: The study aimed to evaluate the safety and efficacy of HTD1801 in treating primary sclerosing cholangitis (PSC). The results showed that HTD1801 had a significant and sustained effect in reducing alkaline phosphatase (ALP). Furthermore, HTD1801 was generally well tolerated. Therefore, HTD1801 may be a novel approach for the treatment of PSC.
AMERICAN JOURNAL OF GASTROENTEROLOGY
(2022)
Review
Medicine, General & Internal
Claudiu Marinel Ionele, Adina Turcu-Stiolica, Mihaela Simona Subtirelu, Bogdan Silviu Ungureanu, George Ovidiu Cioroianu, Ion Rogoveanu
Summary: The association between primary sclerosing cholangitis (PSC) and metabolic bone disease is still unclear. This meta-analysis found no difference in bone mineral density (BMD-LS) between PSC patients and healthy controls, but lower BMD-LS in PSC patients compared to primary biliary cirrhosis (PBC) patients. The lumbar spine T-score was higher in PSC patients compared with PBC patients. Further well-designed and larger-scale studies are needed to confirm these findings.
JOURNAL OF CLINICAL MEDICINE
(2022)
Article
Nutrition & Dietetics
Maryam Ebadi, Elora Rider, Catherine Tsai, Sarah Wang, Ellina Lytvyak, Andrew Mason, Aldo J. Montano-Loza
Summary: This study aimed to investigate the association between severe vitamin D deficiency and disease progression, hepatobiliary malignancies, liver-related mortality, and the need for liver transplantation in primary sclerosing cholangitis (PSC). The results showed that severe vitamin D deficiency was related to an increased risk of hepatobiliary malignancies, and a severe deficiency at diagnosis and persistent deficiencies over time were independently associated with poor clinical liver outcomes.
Review
Gastroenterology & Hepatology
Michal Prokopic, Ulrich Beuers
Summary: PSC is a rare liver disease characterized by bile duct abnormalities leading to liver fibrosis and potential cholangiocarcinoma. The exact pathogenesis is still unclear, with strong association with inflammatory bowel disease. Current medical treatments have not been proven effective, with liver transplantation remaining the most viable option for late-stage disease.
HEPATOLOGY INTERNATIONAL
(2021)
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
Biochemistry & Molecular Biology
Baorong Jiang, Gehui Yuan, Jialin Wu, Qian Wu, Lei Li, Ping Jiang
Summary: Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by bile duct inflammation and dysbiosis of gut microbiota. The abundance of Prevotella copri (P. copri) is significantly decreased in PSC patients, suggesting a potential protective effect. In this study, a PSC mouse model was established and intervention with P. copri was found to improve cholestasis and liver fibrosis by enhancing the FXR-related signalling pathway. This suggests that P. copri intervention may be a potential biological treatment for PSC.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
(2022)
Article
Gastroenterology & Hepatology
Kristoffer Kjaergaard, Kim Frisch, Michael Sorensen, Ole Lajord Munk, Alan Frederick Hofmann, Jacob Horsager, Anna Christina Schacht, Mary Erickson, David Shapiro, Susanne Keiding
Summary: The study demonstrated that OCA increased the hepatic transport of conjugated bile acids in PBC patients, reducing the time hepatocytes are exposed to potentially toxic bile acids.
JOURNAL OF HEPATOLOGY
(2021)
Review
Biochemistry & Molecular Biology
Ji-Won Park, Jung-Hee Kim, Sung-Eun Kim, Jang Han Jung, Myoung-Kuk Jang, Sang-Hoon Park, Myung-Seok Lee, Hyoung-Su Kim, Ki Tae Suk, Dong Joon Kim
Summary: Cholangiopathies are various biliary diseases that affect the biliary epithelium and can lead to cholestasis and liver cirrhosis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most important progressive cholangiopathies in adults, with research focusing on genetic risk, epigenetic changes, dysregulated mucosal immunity, and altered biliary epithelial cell function. Understanding the molecular pathogenesis of these diseases can help clinicians prevent and treat them more effectively.
Article
Gastroenterology & Hepatology
Daniel E. Radford-Smith, Emmanuel A. Selvaraj, Rory Peters, Michael Orrell, Jonathan Bolon, Daniel C. Anthony, Michael Pavlides, Kate Lynch, Alessandra Geremia, Adam Bailey, Emma L. Culver, Fay Probert
Summary: Serum metabolomic profiling has the potential to accurately distinguish between PSC and IgG4-SC, improve the diagnosis of IgG4-RD, and differentiate IgG4-SC from PSC.
LIVER INTERNATIONAL
(2022)
Article
Gastroenterology & Hepatology
Olivier Chazouilleres, Pascal Potier, Charlotte Bouzbib, Bertrand Hanslik, Alexandra Heurgue, Eric NGuyen-Khac, Jerome Gournay, Florence Tanne, Christophe Bureau, Marc Bourliere, Nathalie Ganne-Carrie, Victor de Ledinghen
Summary: Primary sclerosing cholangitis (PSC) is a rare and chronic cholestatic liver disease commonly associated with inflammatory bowel disease (IBD) and characterized by progressive fibro-inflammation of the biliary tree. Non-invasive methods, such as MR cholangiography, play a major role in the diagnosis and monitoring of PSC, while invasive methods like ERCP are important for therapeutic purposes or in suspected cholangiocarcinoma cases.
CLINICS AND RESEARCH IN HEPATOLOGY AND GASTROENTEROLOGY
(2021)
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
Palak J. Trivedi, Andrew J. Muir, Cynthia Levy, Christopher L. Bowlus, Michael P. Manns, Xiaomin Lu, Gerald Crans, Chuhan Chung, G. Mani Subramanian, Robert P. Myers, Zachary Goodman, Naga Chalasani, John M. Vierling, Indra Neil Guha, Gideon M. Hirschfield
Summary: The study found significant inter- and intra-individual variations in serum ALP activity among PSC patients, but it did not correlate with disease progression over a 2-year period. In contrast, variations in ELF scores were smaller and scores measured at multiple timepoints were associated with fibrosis progression and development of cirrhosis.
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY
(2021)
Article
Gastroenterology & Hepatology
Damjana Bogatic, Robert Bryant, Kate D. Lynch, Samuel P. Costello
Summary: Primary sclerosing cholangitis (PSC) is a progressive liver disease with poor prognosis and no effective therapies to prevent progression. This study evaluated potential medical therapies that may exert their effects in PSC by modulation of the gut-liver axis.
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2023)
Review
Gastroenterology & Hepatology
Natassia Tan, John Lubel, William Kemp, Stuart Roberts, Ammar Majeed
Summary: Primary sclerosing cholangitis (PSC) is an orphan, cholestatic liver disease characterized by inflammatory biliary strictures. The pathophysiology of PSC is poorly understood, but it is likely driven by immune dysregulation, gut dysbiosis, and environmental exposures. Currently, there is no proven medical therapy to alter disease progression in PSC, and liver transplantation is the only option for end-stage liver disease. Manipulation of the gut microbiome and bile acid metabolism show promise in phase II trials. Statins and aspirin may reduce the risk of malignancy in PSC patients. Balloon dilatation is safer and equally effective compared to biliary stent insertion for clinically relevant strictures.
JOURNAL OF CLINICAL AND TRANSLATIONAL HEPATOLOGY
(2023)
Article
Gastroenterology & Hepatology
Nora Cazzagon, Sanaa El Mouhadi, Quentin Vanderbecq, Carlos Ferreira, Sarah Finnegan, Sara Lemoinne, Christophe Corpechot, Olivier Chazouilleres, Lionel Arrive
Summary: In individuals with primary sclerosing cholangitis (PSC), novel objective quantitative MRCP metrics provided by MRCP+ were found to correlate with biochemical, elastographic, and radiological prognostic scores and predict adverse outcome-free survival.
