Article
Gastroenterology & Hepatology
Caroline Voskens, Diane Stoica, Marita Rosenberg, Francesco Vitali, Sebastian Zundler, Marion Ganslmayer, Heike Knott, Manuel Wiesinger, Jutta Wunder, Mirko Kummer, Britta Siegmund, Elisabeth Schnoy, Timo Rath, Arndt Hartmann, Holger Hackstein, Beatrice Schuler-Thurner, Carola Berking, Gerold Schuler, Raja Atreya, Markus F. Neurath
Summary: This study reports a successful case of adoptive transfer of ex vivo expanded Tregs in a patient with refractory UC, showing positive responses. These findings suggest that adoptive Treg therapy might be effective in refractory UC.
Article
Immunology
Ramon Cortez, Luana N. Moreira, Marina Padilha, Mariana D. Bibas, Ricardo K. Toma, Gilda Porta, Carla R. Taddei
Summary: Studies have shown that the composition of intestinal microbiome in pediatric patients with primary sclerosing cholangitis (PSC) is influenced by disease, age, and disease activity status, highlighting significant differences in microbiome between PSC patients. Specific genera Veillonella and Megasphaera have increased relative abundance depending on age at diagnosis.
FRONTIERS IN IMMUNOLOGY
(2021)
Review
Gastroenterology & Hepatology
Teresa Da Cunha, Haleh Vaziri, George Y. Wu
Summary: Primary sclerosing cholangitis is a rare disease often associated with inflammatory bowel disease. The pathogenesis may involve changes in cholangiocyte function, gut microbiome, human leukocyte antigen haplotypes, and immune system dysregulation, but these fail to explain the association with inflammatory bowel disease.
JOURNAL OF CLINICAL AND TRANSLATIONAL HEPATOLOGY
(2022)
Article
Multidisciplinary Sciences
Younghun Y. Han, Jinyoung J. Byun, Catherine Zhu, Ryan R. Sun, Julia Y. Roh, Heather Cordell, Hyun-Sung A. Lee, Vikram R. Shaw, Sung Wook Kang, Javad Razjouyan, Matthew A. Cooley, Manal M. Hassan, Katherine A. Siminovitch, Trine Folseraas, David Ellinghaus, Annika Bergquist, Simon M. Rushbrook, Andre Franke, Tom H. Karlsen, Konstantinos N. Lazaridis, Katherine A. McGlynn, Lewis R. Roberts, Christopher Amos, Christoph Schramm, David Shapiro, Elizabeth Goode
Summary: This study used multitrait joint analyses to explore the genetic contribution and correlations between various clinical and epidemiological traits associated with PSC. Several new risk loci and potential susceptibility genes such as MANBA and IRF5 were identified, and in silico drug screening provided candidate agents for further investigation of pharmacological effects in PSC.
NATURE COMMUNICATIONS
(2023)
Review
Immunology
Xinhe Zhang, Xuyong Lin, Xuedan Li, Lin Guan, Yiling Li, Ningning Wang
Summary: This case report describes a patient with ulcerative colitis, primary biliary cholangitis, and autoimmune hepatitis, ultimately diagnosed with overlap syndrome. The accurate diagnosis of overlap syndrome requires multiple laboratory tests and pathological examinations.
FRONTIERS IN IMMUNOLOGY
(2023)
Article
Gastroenterology & Hepatology
In Sub Han, Dong Hoon Baek, Seung Min Hong, Bong Eun Lee, Moon Won Lee, Gwang Ha Kim, Geun Am Song
Summary: The purpose of this study was to estimate the incidence of primary sclerosing cholangitis in patients with ulcerative colitis (UC-PSC) and investigate adverse clinical events associated with UC-PSC. The results showed that patients with UC-PSC had higher healthcare utilization, medication use, surgery rate, and risk of colorectal cancer, biliary tract cancer, and death compared to patients with UC-alone.
INTERNATIONAL JOURNAL OF COLORECTAL DISEASE
(2023)
Article
Immunology
Manon de Krijger, Ishtu L. Hageman, Andrew Y. F. Li Yim, Jan Verhoeff, Juan J. Garcia Vallejo, Patricia H. P. van Hamersveld, Evgeni Levin, Theodorus B. M. Hakvoort, Manon E. Wildenberg, Peter Henneman, Cyriel Y. Ponsioen, Wouter J. de Jonge
Summary: Distinguishing patients with PSC-UC from those with UC alone is possible through peripheral blood DNA methylation, indicating a potential for biomarker development.
FRONTIERS IN IMMUNOLOGY
(2022)
Review
Oncology
G. S. Sica, B. Sensi, L. Siragusa, F. Blasi, B. Crispino, B. Pirozzi, R. Angelico, L. Biancone, J. Khan
Summary: Colon cancer in ulcerative colitis patients with liver transplant (UCCOLT) due to primary sclerosing cholangitis poses significant treatment challenges. Proctocolectomy and end-ileostomy (PC) and restorative ileal pouch-anal anastomosis (IPAA) have shown good results in these patients, but ileo-rectal anastomosis (IRA) may also be considered in selected cases.
Article
Gastroenterology & Hepatology
Aiva Lundberg Bave, Annika Bergquist, Matteo Bottai, Anna Warnqvist, Erik von Seth, Caroline Nordenvall
Summary: The study found that PSC patients have a four times overall increased risk of developing cancer compared to the general population, with a particularly higher risk of developing hepatobiliary, colorectal, and pancreatic cancer.
HEPATOLOGY INTERNATIONAL
(2021)
Article
Gastroenterology & Hepatology
Timur Liwinski, Sina Huebener, Lara Henze, Peter Huebener, Melina Heinemann, Marcus Tetzlaff, Marie Hiller, Bettina Jagemann, Rambabu Surabattula, Diana Leeming, Morten Karsdal, Erika Monguzzi, Guido Schachschal, Thomas Roesch, Corinna Bang, Andre Franke, Ansgar W. Lohse, Detlef Schuppan, Christoph Schramm
Summary: This study suggests that a short-term gluten/ATI-free diet may not lead to clinical improvement in patients with PSC-IBD, but it can improve biomarkers of intestinal inflammation and barrier function, as well as affect changes in the enteric microbiota.
ALIMENTARY PHARMACOLOGY & THERAPEUTICS
(2023)
Article
Gastroenterology & Hepatology
Aiva Lundberg Bave, Ola Olen, Jonas Soederling, Jonas F. Ludvigsson, Annika Bergquist, Caroline Nordenvall
Summary: Colectomy in UC patients was not associated with a decreased risk of subsequent PSC. The observed differences in the risk of PSC development over calendar periods are likely due to changes in PSC-diagnosis and UC-treatment.
UNITED EUROPEAN GASTROENTEROLOGY JOURNAL
(2023)
Review
Gastroenterology & Hepatology
Johannes R. Hov, Tom H. Karlsen
Summary: This Review explores the links between Primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD), and the mechanisms related to the gut-liver axis. It is found that the gut microbiota in people with PSC differs from that of healthy individuals, but the specific microbial factors that differentiate PSC from IBD without PSC are poorly understood. The potential existence of a specific unknown factor related to the gut-liver axis as a cause of PSC is postulated.
NATURE REVIEWS GASTROENTEROLOGY & HEPATOLOGY
(2023)
Article
Cell Biology
Xiaojun Jiang, Annika Bergquist, Britt-Sabina Loscher, Geetha Venkatesh, Jeff E. Mold, Kristian Holm, Jon K. Laerdahl, Sigrid S. Skanland, Kimia T. Maleki, Martin Cornillet, Kjetil Tasken, Andre Franke, Tom H. Karlsen, Niklas K. Bjorkstrom, Espen Melum
Summary: A heterozygous germline missense mutation in the CD100 gene was found in a family with PSC, leading to T cell functional defects and increased proliferation as well as impaired interferon-gamma production. Mice with homologous mutation showed a higher susceptibility to develop severe cholangitis, which could be attenuated by transferring wild-type T cells. The study suggests a potential therapeutic role for T cells in protecting against PSC.
SCIENCE TRANSLATIONAL MEDICINE
(2021)
Article
Gastroenterology & Hepatology
Henry E. Pratt, Tong Wu, Shaimae Elhajjajy, Jeffrey Zhou, Kate Fitzgerald, Tom Fazzio, Zhiping Weng, Daniel S. Pratt
Summary: Noncoding PSC-risk variants are enriched within immune-specific enhancers, particularly ones involved in T-cell response to antigenic stimulation. Regulatory elements play a key role in the pathophysiology of PSC.
HEPATOLOGY COMMUNICATIONS
(2023)
Article
Gastroenterology & Hepatology
Sjoukje-Marije Haisma, Rinse K. Weersma, Maria E. Joosse, Barbara A. E. de Koning, Tim de Meij, Bart G. P. Koot, Victorien Wolters, Obbe Norbruis, Mark J. Daly, Christine Stevens, Ramnik J. Xavier, Jukka Koskela, Manuel A. Rivas, Marijn C. Visschedijk, Henkjan J. Verkade, Ruggero Barbieri, Dianne B. H. Jansen, Eleonora A. M. Festen, Patrick F. van Rheenen, Cleo C. van Diemen
Summary: Whole-exome sequencing was performed on 29 patient-parent trios with early-onset PSC, identifying at least one potentially pathogenic variant in 22 trios. A total of 36 candidate risk genes were prioritized, with variants affecting genes involved in transmembrane transport, immune response, and epithelial barrier function. Further validation and functional evaluation of these genes are required to establish their causal role in early-onset PSC.
LIVER INTERNATIONAL
(2021)