Article
Gastroenterology & Hepatology
Toshihiko Arizumi, Susumu Tazuma, Hiroyuki Isayama, Takahiro Nakazawa, Toshio Tsuyuguchi, Hajime Takikawa, Atsushi Tanaka
Summary: This study investigated the association between UDCA treatment and improved LT-free survival in a cohort of Japanese PSC patients.
JOURNAL OF GASTROENTEROLOGY
(2022)
Article
Gastroenterology & Hepatology
John E. Eaton, Abdul Haseeb, Christian Rupp, Leonardo H. Eusebi, Kim Munster, Robert Voitl, Douglas Thorburn, Cyriel Y. Ponsioen, Felicity T. Enders, Bret T. Petersen, Barham K. Abu Dayyeh, Todd H. Baron, Vinay Chandrasekhara, Christopher J. Gostout, Michael J. Levy, John Martin, Andrew C. Storm, Ross Dierkhising, Patrick S. Kamath, Gregory J. Gores, Mark Topazian
Summary: The resolution of jaundice after endoscopic treatment of biliary strictures in patients with primary sclerosing cholangitis (PSC) is associated with longer transplant-free survival. Predictors of jaundice resolution include age, duration of jaundice, Mayo Risk Score, and the location of the most advanced biliary stricture. Other factors, such as higher Mayo Risk Score at the time of ERCP, lower pre-ERCP total serum bilirubin, and persistence of jaundice after endoscopic therapy, may predict the risk of death or transplant in patients with PSC.
HEPATOLOGY COMMUNICATIONS
(2022)
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
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
Gastroenterology & Hepatology
Karolina M. Wronka, Emil Bik, Piotr Milkiewicz
Summary: This study assessed the maternal and fetal outcomes of pregnancy in patients with primary sclerosing cholangitis (PSC). The results showed that pregnancy in PSC patients appears to be well tolerated, but requires close monitoring by an obstetrician and an experienced hepatologist.
DIGESTIVE AND LIVER DISEASE
(2022)
Article
Gastroenterology & Hepatology
Laura Cristoferi, Marco Porta, Davide Paolo Bernasconi, Filippo Leonardi, Alessio Gerussi, Giacomo Mulinacci, Andrea Palermo, Camilla Gallo, Miki Scaravaglio, Eliana Stucchi, Cesare Maino, Davide Ippolito, Daphne D'Amato, Carlos Ferreira, Alessandra Nardi, Rajarshi Banerjee, Maria Grazia Valsecchi, Laura Antolini, Rocco Corso, Sandro Sironi, Stefano Fagiuoli, Pietro Invernizzi, Marco Carbone
Summary: Magnetic resonance cholangiopancreatography (MRCP) is the gold standard for diagnosing primary sclerosing cholangitis (PSC). This study evaluated the prognostic performance of quantitative MRCP metrics in PSC and found that the number of biliary features was associated with a higher risk of hepatobiliary complications in PSC patients. The quantitative MRCP-derived PSC (qMRCP-PSC) score showed better predictive ability and may serve as a potential surrogate endpoint for clinical trials.
DIGESTIVE AND LIVER DISEASE
(2023)
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.
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)
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
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
Medicine, General & Internal
Fuat H. Saner, Alexandra Frey, Bjoern-Ole Stueben, Dieter P. Hoyer, Katharina Willuweit, Martina Daniel, Jassin Rashidi-Alavieh, Jurgen W. Treckmann, Hartmut H. Schmidt
Summary: In this retrospective study of 82 patients who underwent liver transplantation for primary sclerosing cholangitis (PSC), the authors assessed morbidity, survival rates, PSC recurrence, and the impact of donor characteristics in long-term follow-up. The study showed that PSC patients had excellent 10-year survival rates and a lower lab-MELD score was associated with longer survival. Donor characteristics did not affect patient survival.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Gastroenterology & Hepatology
Enni Vanhanen, Ali Ovissi, Kaija-Leena Kolho, Andrea Tenca
Summary: The aim of this study was to evaluate the diagnostic accuracy of MRCP in the diagnosis of DS in patients with pediatric-onset PSC. The sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, and accuracy of MRCP for detecting DS were calculated. The study found that the diameter limits of DS should probably be less strict in MRCP than in ERCP.
DIGESTIVE AND LIVER DISEASE
(2023)
Article
Cell Biology
Christine L. Zimmer, Erik von Seth, Marcus Buggert, Otto Strauss, Laura Hertwig, Son Nguyen, Alicia Y. W. Wong, Chiara Zotter, Lena Berglin, Jakob Michaelsson, Marcus Reuterwall Hansson, Urban Arnelo, Ernesto Sparrelid, Ewa C. S. Ellis, Johan D. Soderholm, Asa Keita, Kristian Holm, Volkan Ozenci, Johannes R. Hov, Jeff E. Mold, Martin Cornillet, Andrea Ponzetta, Annika Bergquist, Niklas K. Bjorkstrom
Summary: The study on patients with primary sclerosing cholangitis (PSC) revealed the characteristics of the human biliary immunological landscape, highlighting differences in immune cell composition compared to blood and the pathological features of biliary inflammation in PSC patients.
SCIENCE TRANSLATIONAL MEDICINE
(2021)