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primary sclerosing cholangitis ulcerative colitis liver transplantation

Tobias J Weismüller, Palak J Trivedi, Annika Bergquist, Mohamad Imam, Henrike Lenzen, Cyriel Y Ponsioen, Kristian Holm, Daniel Gotthardt, Martti A Färkkilä, Hanns-Ulrich Marschall, Douglas Thorburn, Rinse K Weersma, Johan Fevery, Tobias Mueller, Olivier Chaouillères, Kornelius Schulze, Konstantinos N Lazaridis, Sven Almer, Stephen P Pereira, Cynthia Levy, Andrew Mason, Sigrid Naess, Christopher L Bowlus, Annarosa Floreani, Emina Halilbasic, Kidist K Yimam, Piotr Milkiewicz, Ulrich Beuers, Dep K Huynh, Albert Pares, Christine N Manser, George N Dalekos, Bertus Eksteen, Pietro Invernizzi, Christoph P Berg, Gabi I Kirchner, Christoph Sarrazin, Vincent Zimmer, Luca Fabris, Felix Braun, Marco Marzioni, Brian D Juran, Karouk Said, Christian Rupp, Kalle Jokelainen, Maria Benito de Valle, Francesca Saffioti, Angela Cheung, Michael Trauner, Christoph Schramm, Roger W Chapman, Tom H Karlsen, Erik Schrumpf, Christian P Strassburg, Michael P Manns, Keith D Lindor, Gideon M Hirschfield, Bettina E Hansen, Kirsten M Boberg
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large, international cohort of patients with PSC. METHODS: We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries...
March 5, 2017: Gastroenterology
Martin Blaho, Petr Dítě, Martina Bojková, Martin Rydlo, Tomáš Kupka, Pavel Svoboda, Pavel Klvaňa, Arnošt Martínek
Sclerosing cholangitides represent a group of chronic biliary obstructive diseases which include primary sclerosing cholangitis (PSC), IgG4 associated sclerosing cholangitis (IgG4-SC) and secondary sclerosing cholangitis (SSC). The manifestations of the diseases are similar, but their asymptomatic course is also frequent. IgG4-SC belongs to the group of IgG4 associated diseases and it is the most frequently related to type 1 autoimmune pancreatitis. Diagnosing of IgG4-SC is based on typical histopathological images, shape changes revealed by diagnostic imaging, serological tests, concurrent impairment of other organs and response to therapy, where IgG4-SC responds well to treatment with corticoids, whereas the only possibility for the remaining units is endoscopic intervention or liver transplantation...
2017: Vnitr̆ní Lékar̆ství
Ahmad H Ali, Elizabeth J Carey, Keith D Lindor
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with detrimental sequela. In many patients, PSC progresses to end-stage liver disease and hepatobiliary cancer. There is no medical therapy that is proven to halt or reverse the progression of PSC. Approximately 70 to 80% of PSC patients have inflammatory bowel disease, usually ulcerative colitis. The etiology of PSC is poorly understood. Several lines of evidence suggest that the intestinal microbiota plays an important role in the etiopathogenesis of PSC...
September 2016: Seminars in Liver Disease
A J Montano-Loza, R A Bhanji, S Wasilenko, A L Mason
BACKGROUND: Autoimmune liver diseases (AILD) constitute the third most common indication for liver transplantation (LT) worldwide. Outcomes post LT are generally good but recurrent disease is frequently observed. AIMS: To describe the frequency and risk factors associated with recurrent AILD post-LT and provide recommendations to reduce the incidence of recurrence based on levels of evidence. METHODS: A systematic review was performed for full-text papers published in English-language journals, using the keywords 'autoimmune hepatitis (AIH)', 'primary biliary cholangitis and/or cirrhosis (PBC)', 'primary sclerosing cholangitis (PSC)', 'liver transplantation' and 'recurrent disease'...
February 2017: Alimentary Pharmacology & Therapeutics
Montserrat Fraga, Nicolas Fournier, Ekaterina Safroneeva, Valérie Pittet, Sébastien Godat, Alex Straumann, Andreas Nydegger, Stephan R Vavricka, Darius Moradpour, Alain M Schoepfer
BACKGROUND AND AIM: Primary sclerosing cholangitis (PSC) represents the most common hepatobiliary extraintestinal manifestation of inflammatory bowel disease (IBD). We aimed to assess the prevalence of PSC in the Swiss Inflammatory Bowel Disease Cohort Study, to identify associated risk factors, and to describe the long-term evolution. PATIENTS AND METHODS: Data of patients enrolled into the Swiss Inflammatory Bowel Disease Cohort Study were analyzed. Logistic regression modeling was performed to identify risk factors for PSC...
January 2017: European Journal of Gastroenterology & Hepatology
Andrea Tenca, Martti Färkkilä, Johanna Arola, Tytti Jaakkola, Roberto Penagini, Kaija-Leena Kolho
BACKGROUND: The natural history of pediatric-onset primary sclerosing cholangitis (PSC) and overlap with autoimmune hepatitis (PSC/AIH) is poorly known. OBJECTIVE: The aim of this study was to evaluate the clinical outcome of patients with pediatric-onset disease in a tertiary referral center. METHODS: We traced 33 patients (median age at diagnosis 16 years), with PSC or PSC/AIH in cholangiography and liver histology diagnosed between December 1993 and 2011, at Helsinki University Hospital...
August 2016: United European Gastroenterology Journal
Pamela L Valentino, Shanna Wiggins, Sarah Harney, Roshan Raza, Christine K Lee, Maureen M Jonas
OBJECTIVES: Data regarding pediatric primary sclerosing cholangitis (PSC) natural history are limited. We describe a large pediatric PSC cohort with longitudinal follow-up. METHODS: The present study records review of pediatric patients with PSC diagnosed between 1984 and 2014. RESULTS: N = 120 (63% M) ages 1 to 21 years (median 14 years) at diagnosis. 27% (31/113) had autoimmune sclerosing cholangitis (ASC), 24% had exclusive small duct PSC, METAVIR stage was F3-F4 in 41%...
December 2016: Journal of Pediatric Gastroenterology and Nutrition
Irene Franceschet, Nora Cazzagon, Teresa Del Ross, Renata D'Incà, Alessandra Buja, Annarosa Floreani
BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with a strong association with inflammatory bowel disease (IBD). Medical treatment for PSC is still disappointing, whereas immunomodulators and biologics have been proven to be effective in IBD. AIMS: This study aimed to analyze (i) the natural history of patients with PSC with or without IBD and (ii) the long-term efficacy of biologics in patients with PSC and concomitant IBD or rheumatological disorders...
May 2016: European Journal of Gastroenterology & Hepatology
Jisun Yoon, Seak Hee Oh, Hyun Jin Kim, Sang Hyoung Park, Byong Duk Ye, Suk-Kyun Yang, Kyung Mo Kim
PURPOSE: Primary sclerosing cholangitis (PSC) is a rare condition that can be associated with inflammatory bowel disease (IBD). The aim of this study was to evaluate PSC and its association with IBD in children. METHODS: We retrospectively enrolled 13 pediatric patients (<18 years) with PSC treated at Asan Medical Center between June 1989 and December 2013. Clinical findings and long-term outcomes were investigated. During the same period, the incidence of PSC among IBD patients was evaluated among 600 Crohn disease (CD) and 210 ulcerative colitis (UC) patients...
December 2015: Pediatric Gastroenterology, Hepatology & Nutrition
J Fevery, W Van Steenbergen, J Van Pelt, W Laleman, I Hoffman, K Geboes, S Vermeire, F Nevens
BACKGROUND: Approximately 20% of primary sclerosing cholangitis (PSC) patients with concomitant inflammatory bowel disease (IBD) have Crohn's disease (CD). AIM: To compare PSC/CD with other PSC patients. METHODS: Retrospective study of 240 PSC patients diagnosed between 1975 and 2012 (median follow-up 12 years). Activity of PSC at diagnosis was assessed by liver biopsy, Mayo risk and ERC scores. Survival without liver transplantation, number of transplantations and liver-related death were endpoints...
March 2016: Alimentary Pharmacology & Therapeutics
F Åberg, A Abdulle, A Mäkelä, M Nissinen
Guidelines recommend colonoscopy screening for possible asymptomatic inflammatory bowel disease (IBD) in all patients diagnosed with primary sclerosing cholangitis (PSC). PSC-IBD warrants regular dysplasia-surveillance colonoscopy. However, no consensus exists regarding follow-up colonoscopy in PSC patients without IBD who remain asymptomatic. We describe a 43-year-old female who had undergone liver transplantation (LT) due to advanced PSC. Previous colonoscopies had been normal. The post-transplantation course was uneventful, with no rejections and signs of PSC recurrence...
November 2015: Transplantation Proceedings
Giulia Bonato, Laura Cristoferi, Mario Strazzabosco, Luca Fabris
UNLABELLED: Primary sclerosing cholangitis (PSC) is a chronic inflammatory liver disease of unknown etiology, primarily targeting cholangiocytes at any portion of the biliary tree. No effective medical treatments are currently available. A unique feature of PSC is its close association (about 80%) with inflammatory bowel disease (IBD), mainly ulcerative colitis (UC). As in many chronic inflammatory conditions, cancer development can complicate PSC, accounting for >40% of deaths. Cholangiocarcinoma (CCA), gallbladder carcinoma (GBC) and colorectal carcinoma (CRC) have been variably associated to PSC, with a prevalence up to 13-14%...
2015: Digestive Diseases
Roberta E Rossi, Dario Conte, Sara Massironi
Primary sclerosing cholangitis (PSC) is a chronic progressive disease, usually associated with underlying inflammatory bowel diseases (IBDs), with a prevalence of 60-80% in western countries. Herein, we review the current knowledge about the association between PSC and IBD in terms of clinical approach and long-term patient management. A PubMed search was conducted for English-language publications from 2000 through 2015 using the following keywords: primary sclerosing cholangitis, inflammatory bowel disease, ulcerative colitis, Crohn's disease, diagnosis, therapy, follow-up, and epidemiology...
February 2016: European Journal of Gastroenterology & Hepatology
Anna M Banc-Husu, Sudha A Anupindi, Henry C Lin
Hepatic artery thrombosis (HAT) is a serious complication after liver transplantation. This is the first report of spontaneous resolution of HAT in pediatric liver transplant patients on low molecular weight heparin therapy. A total of 2 patients, a 26-month-old boy who presented with acute liver failure and required emergent liver transplantation and a 15-year-old boy with ulcerative colitis and autoimmune hepatitis-primary sclerosing cholangitis overlap underwent liver transplantation for progressive cirrhosis; both developed HAT during the postoperative period...
April 2016: Journal of Pediatric Gastroenterology and Nutrition
Tatiana Hildebrand, Nadine Pannicke, Alexander Dechene, Daniel N Gotthardt, Gabriele Kirchner, Florian P Reiter, Martina Sterneck, Kerstin Herzer, Henrike Lenzen, Christian Rupp, Hannelore Barg-Hock, Philipp de Leuw, Andreas Teufel, Vincent Zimmer, Frank Lammert, Christoph Sarrazin, Ulrich Spengler, Christian Rust, Michael P Manns, Christian P Strassburg, Christoph Schramm, Tobias J Weismüller
Liver transplantation (LT) is the only definitive treatment for patients with end-stage liver disease due to primary sclerosing cholangitis (PSC), but a high rate of biliary strictures (BSs) and of recurrent primary sclerosing cholangitis (recPSC) has been reported. In this multicenter study, we analyzed a large patient cohort with a long follow-up in order to evaluate the incidence of BS and recPSC, to assess the impact on survival after LT, and to identify risk factors. We collected clinical, surgical, and laboratory data and records on inflammatory bowel disease (IBD), immunosuppression, recipient and graft outcome, and biliary complications (based on cholangiography and histology) of all patients who underwent LT for PSC in 10 German transplant centers between January 1990 and December 2006; 335 patients (68...
January 2016: Liver Transplantation
Ahmad Khosravi Khorashad, Mohammad Khajedaluee, Elham Mokhtari Amirmajdi, Ali Bahari, Mohammad Reza Farzanehfar, Mitra Ahadi, Siavash Abedini, Mohammad Reza Abdollahi, Rosita Vakili, Hassan Vossoughi Nia
AIM: To identify primary sclerosing cholangitis (PSC) predisposing factors in order to prevent inflammatory bowel disease (IBD) progression to PSC. BACKGROUND: IBD is commonly associated with PSC and there is no effective therapy for PSC except for liver transplantation. PATIENTS AND METHODS: This retrospective study was conducted on 447 IBD patients from IBD Clinics of Ghaem and Emam Reza Hospitals. Data were collected by interview and through a review of the patients' medical records...
2015: Gastroenterology and Hepatology From Bed to Bench
Fabian Schnitzler, Matthias Friedrich, Johannes Stallhofer, Ulf Schönermarck, Michael Fischereder, Antje Habicht, Nazanin Karbalai, Christiane Wolf, Marianne Angelberger, Torsten Olszak, Florian Beigel, Cornelia Tillack, Burkhard Göke, Reinhart Zachoval, Gerald Denk, Markus Guba, Christian Rust, Norbert Grüner, Stephan Brand
BACKGROUND: Currently, limited data of the outcome of inflammatory bowel disease (IBD) in patients after solid organ transplantation (SOT) are available. We aimed to analyze effects of SOT on the IBD course in a large IBD patient cohort. METHODS: Clinical data from 1537 IBD patients were analyzed for patients who underwent SOT (n = 31) between July 2002 and May 2014. Sub-analyses included SOT outcome parameters, IBD activity before and after SOT, and efficacy of IBD treatment...
2015: PloS One
Reena Ravikumar, Emmanuel Tsochatzis, Sophie Jose, Michael Allison, Anuja Athale, Felicity Creamer, Bridget Gunson, Vikram Iyer, Mansoor Madanur, Derek Manas, Andrea Monaco, Darius Mirza, Nicola Owen, Keith Roberts, Gourab Sen, Parthi Srinivasan, Stephen Wigmore, Giuseppe Fusai, Bimbi Fernando, Andrew Burroughs
BACKGROUND & AIMS: The association between primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) is well recognised. However, the relationship between IBD and recurrent PSC (rPSC) is less well understood. We assessed the prevalence of rPSC and analysed the factors associated with rPSC post-liver transplantation and its influence on graft and patient survival. METHODS: This is a UK multicentre observational cohort study across six of the seven national liver transplant units...
November 2015: Journal of Hepatology
Ersilia M DeFilippis, Sonal Kumar
Nearly one-third of patients with inflammatory bowel disease (IBD) have abnormal liver tests, which can be indicative of underlying hepatic disease. Primary sclerosing cholangitis has a clear association with ulcerative colitis, but other autoimmune disorders such as autoimmune hepatitis (AIH) have also been associated with IBD. AIH may also occur in the setting of an overlap syndrome or in the setting of medications, particularly tumor necrosis factor alpha inhibitors. Importantly, some studies have shown that IBD patients with AIH fail treatment more frequently than IBD patients without AIH...
October 2015: Digestive Diseases and Sciences
Mette Vesterhus, Johannes Roksund Hov, Anders Holm, Erik Schrumpf, Ståle Nygård, Kristin Godang, Ina Marie Andersen, Sigrid Naess, Douglas Thorburn, Francesca Saffioti, Morten Vatn, Odd Helge Gilja, Fridtjof Lund-Johansen, Trygve Syversveen, Knut Brabrand, Albert Parés, Cyriel Y Ponsioen, Massimo Pinzani, Martti Färkkilä, Bjørn Moum, Thor Ueland, Helge Røsjø, William Rosenberg, Kirsten Muri Boberg, Tom H Karlsen
UNLABELLED: There is a need to determine biomarkers reflecting disease activity and prognosis in primary sclerosing cholangitis (PSC). We evaluated the prognostic utility of the enhanced liver fibrosis (ELF) score in Norwegian PSC patients. Serum samples were available from 305 well-characterized large-duct PSC patients, 96 ulcerative colitis patients, and 100 healthy controls. The PSC patients constituted a derivation panel (recruited 1992-2006 [n = 167]; median age 41 years, 74% male) and a validation panel (recruited 2008-2012 [n = 138]; median age 40 years, 78% male)...
July 2015: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
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