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Chen Y, Chen R, Li H, Shuai Z. Clinical management of autoimmune liver diseases: juncture, opportunities, and challenges ahead. Immunol Res 2025; 73:67. [PMID: 40195209 PMCID: PMC11976385 DOI: 10.1007/s12026-025-09622-9] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/27/2024] [Accepted: 03/14/2025] [Indexed: 04/09/2025]
Abstract
The three major autoimmune liver diseases are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).These conditions are assumed to result from a breakdown in immunological tolerance, which leads to an inflammatory process that causes liver damage.The self-attack is started by T-helper cell-mediated identification of liver autoantigens and B-cell production of autoantibodies,and it is maintained by a reduction in the number and activity of regulatory T-cells.Infections and environmental factors have been explored as triggering factors for these conditions, in addition to a genetic predisposition.Allelic mutations in the HLA locus have been linked to vulnerability, as have relationships with single nucleotide polymorphisms in non-HLA genes.Despite the advances in the management of these diseases, there is no curative treatment for these disorders, and a significant number of patients eventually progress to an end-stage liver disease requiring liver transplantation.In this line, tailored immune-therapeutics have emerged as possible treatments to control the disease.In addition, early diagnosis and treatment are pivotal for reducing the long-lasting effects of these conditions and their burden on quality of life.Herein we present a review of the etiology, clinical presentation, diagnosis, and challenges on ALDs and the feasible solutions for these complex diseases.
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MESH Headings
- Humans
- Hepatitis, Autoimmune/therapy
- Hepatitis, Autoimmune/diagnosis
- Hepatitis, Autoimmune/immunology
- Hepatitis, Autoimmune/etiology
- Cholangitis, Sclerosing/therapy
- Cholangitis, Sclerosing/diagnosis
- Cholangitis, Sclerosing/immunology
- Liver Cirrhosis, Biliary/therapy
- Liver Cirrhosis, Biliary/diagnosis
- Liver Cirrhosis, Biliary/immunology
- Animals
- Immunotherapy/methods
- Autoimmune Diseases/therapy
- Autoimmune Diseases/diagnosis
- Disease Management
- Genetic Predisposition to Disease
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Affiliation(s)
- Yangfan Chen
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China
| | - Ruofei Chen
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China
| | - Haiyan Li
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China
| | - Zongwen Shuai
- Department of Rheumatology and Immunology, The First Affiliated Hospital of Anhui Medical University, Hefei, 230022, China.
- Inflammation and Immune Mediated Diseases Laboratory of Anhui Province, Hefei, 230032, China.
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Cançado GGL, Hirschfield GM. Management of primary sclerosing cholangitis: Current state-of-the-art. Hepatol Commun 2024; 8:e0590. [PMID: 39774274 PMCID: PMC11567710 DOI: 10.1097/hc9.0000000000000590] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/18/2024] [Accepted: 10/08/2024] [Indexed: 01/11/2025] Open
Abstract
Primary sclerosing cholangitis is a chronic liver disease characterized by progressive inflammation and fibrosis of medium-large bile ducts, most commonly in association with inflammatory bowel disease. Most patients have a progressive disease course, alongside a heightened risk of hepatobiliary and colorectal cancer. Medical therapies are lacking, and this, in part, reflects a poor grasp of disease biology. As a result, current management is largely supportive, with liver transplantation an effective life-prolonging intervention when needed, but not one that cures disease. Emerging therapies targeting disease progression, as well as symptoms such as pruritus, continue to be explored. The trial design is increasingly cognizant of the application of thoughtful inclusion criteria, as well as better endpoints aimed at using surrogates of disease that can identify treatment benefits early. This is hoped to facilitate much-needed advances toward developing safe and effective interventions for patients.
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Jin H, Fu C, Sun X, Fan C, Chen J, Zhou H, Liu K, Xu H. The assessment of postoperative cholangitis in malignant biliary obstruction: a real-world study of nasobiliary drainage after endoscopic placement of self-expandable metal stent. Front Oncol 2024; 14:1440131. [PMID: 39610922 PMCID: PMC11602394 DOI: 10.3389/fonc.2024.1440131] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/29/2024] [Accepted: 10/29/2024] [Indexed: 11/30/2024] Open
Abstract
Objectives Endoscopic retrograde cholangiopancreatography(ERCP) with endoscopic metallic biliary endoprosthesis(EMBE) serves as a crucial palliative treatment for advanced malignant biliary obstruction(MBO). While endoscopic nasobiliary drainage(ENBD) effectively reduces post-ERCP cholangitis (PEC) incidence, its impact on PEC in MBO patients is unclear. This study evaluates ENBD's effects on PEC in patients undergoing EMBE and identifies risk factors. Methods This retrospective cohort study at the First Hospital of Jilin University involved MBO patients who underwent EMBE from September 2011 to September 2022. Propensity score matching (PSM) was applied to minimize selection bias. Primary and secondary outcomes included the incidence and recovery rate/time of PEC, biliary drainage success, and hospitalization duration. Univariate, multivariate, and Lasso regression analyses identified independent risk factors. Results In this study of 1,008 patients, 730 were analyzed after PSM(365 each in the EMBE+ENBD and EMBE groups). No significant differences were observed in PEC incidence(10.7% vs 11.2%, p=0.9057) or recovery rates(48.7% vs 31.7%, p=0.1855). However, PEC recovery time was shorter in the EMBE+ENBD group(4.0 days [3.0, 6.0] vs 5.0 days [4.0, 7.5], p=0.0240), as was hospitalization duration(6.0 days [4.0, 8.0] vs 7.0 days [5.0, 10.0], p=0.0146), and a higher success rate of biliary drainage(54.0% vs 43.3%, p=0.0049). Tumor location(HR 1.10, 95% CI 1.00-1.20) and preoperative total bilirubin(HR 2.13, 95% CI 1.66-2.73) were identified as independent risk factors. Conclusion In this large-scale PSM study, ENBD did not reduce PEC incidence but expedited recovery and shortened hospital stays. Patients with hilar MBO of Bismuth III-IV or high preoperative bilirubin were more prone to PEC.
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Affiliation(s)
- Hengwei Jin
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Chang Fu
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Xu Sun
- Clinical Medical College, Changchun University of Traditional Chinese Medicine, Changchun, Jilin, China
| | - Changqing Fan
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Junhong Chen
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Hao Zhou
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Kai Liu
- Department of Hepatobiliary and Pancreatic Surgery, General Surgery Center, The First Hospital of Jilin University, Changchun, Jilin, China
| | - Hongji Xu
- Department of Abdominal Surgery, Guiqian International General Hospital, Guiyang, Guizhou, China
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Karsenti D, Gincul R, Belle A, Vienne A, Weiss E, Vanbiervliet G, Gronier O. Antibiotic prophylaxis in digestive endoscopy: Guidelines from the French Society of Digestive Endoscopy. Endosc Int Open 2024; 12:E1171-E1182. [PMID: 39411364 PMCID: PMC11479795 DOI: 10.1055/a-2415-9414] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/13/2024] [Accepted: 09/13/2024] [Indexed: 10/19/2024] Open
Abstract
Digestive endoscopy is a highly dynamic medical discipline, with the recent adoption of new endoscopic procedures. However, comprehensive guidelines on the role of antibiotic prophylaxis in these new procedures have been lacking for many years. The Guidelines Commission of the French Society of Digestive Endoscopy (SFED) convened in 2023 to establish guidelines on antibiotic prophylaxis in digestive endoscopy for all digestive endoscopic procedures, based on literature data up to September 1, 2023. This article summarizes these new guidelines and describes the literature review that fed into them.
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Affiliation(s)
- David Karsenti
- Digestive Endoscopy Unit, Clinique Paris-Bercy, Charenton-le-Pont, France
- Gastroenterology, Centre d'Explorations Digestives, Paris, France
| | - Rodica Gincul
- Department of Gastroenterology, Hôpital Privé Jean Mermoz, Lyon, France
| | - Arthur Belle
- Gastroenterology and Digestive Oncology, Cochin Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France
| | - Ariane Vienne
- Digestive Endoscopy Unit, Hôpital Privé d' Antony, Antony, France
- Digestive Endoscopy Unit, Hôpital Européen Georges Pompidou, Assistance Publique Hôpitaux de Paris, Paris, France
| | - Emmanuel Weiss
- Département d'Anesthésie-Réanimation, Hôpital Beaujon, Clichy, France
| | - Geoffroy Vanbiervliet
- Pôle DIGi-TUNED, Endoscopie Digestive, CHU de Nice, Hôpital L'Archet 2, Nice, France
| | - Olivier Gronier
- Digestive Endoscopy Unit, Clinique Sainte Barbe, Strasbourg, France
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Kim KH, Chon HK, Song TJ, Ahn DW, Lee ES, Lee YN, Lee YS, Jeon TJ, Park CH, Cho KB, Lee DW, Park JS, Yoon SB, Chung KH, Lee J, Choi M. [Quality Indicators of Endoscopic Retrograde Cholangiopancreatography in Korea]. THE KOREAN JOURNAL OF GASTROENTEROLOGY = TAEHAN SOHWAGI HAKHOE CHI 2024; 84:111-122. [PMID: 39319432 DOI: 10.4166/kjg.2024.067] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/24/2024] [Revised: 08/05/2024] [Accepted: 08/05/2024] [Indexed: 09/26/2024]
Abstract
Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure that requires abundant clinical experience and endoscopic skills, and can lead to various complications, some of which may progress to life-threatening conditions. With expanding indications and technological advancements, ERCP is widely utilized, enhancing procedural accessibility. However, without proper quality management, the procedure can pose significant risks. Quality management in ERCP is essential to ensure safe and successful procedures and meet societal demands for improved healthcare competitiveness. To address these concerns, the Korean Society of Pancreatobiliary Endoscopy has developed a Korean-specific ERCP quality indicator reflecting domestic medical environments and realities. Initially, based on a review of foreign ERCP quality indicators and related literatures, key questions were formulated for five pre-procedural items, three intra-procedural items, and four post-procedural items. Descriptions and recommendations for each item were selected through peer evaluation. The developed Korean-specific ERCP quality indicator was reviewed by external experts based on the latest evidence and consensus in this fields. This Korean-specific indicator is expected to significantly contribute to improving ERCP quality in Korea, as it is tailored to local needs.
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Affiliation(s)
- Ki-Hyun Kim
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
| | - Hyung Ku Chon
- Department of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea
| | - Tae Jun Song
- Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong Won Ahn
- Department of Internal Medicine, SMG-SNU Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea
| | - Eaum Seok Lee
- Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea
| | - Yun Nah Lee
- Department of Internal Medicine, SoonChunHyang University School of Medicine, Bucheon, Korea
| | - Yoon Suk Lee
- Department of Internal Medicine, Inje University College of Medicine, Ilsan Paik Hospital, Goyang, Korea
| | - Tae Joo Jeon
- Department of Internal Medicine, Inje University College of Medicine, Sanggye Paik Hospital, Seoul, Korea
| | - Chang Hwan Park
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
| | - Kwang Bum Cho
- Department of Internal Medicine, Keimyung University School of Medicine, Dongsan Medical Center, Daegu, Korea
| | - Dong Wook Lee
- Department of Internal Medicine, Kyungpook National University Chilgok Hospital, School of Medicine, Kyungpook National University, Daegu, Korea
| | - Jin-Seok Park
- Department of Internal Medicine, Inha University School of Medicine, Incheon, Korea
| | - Seung Bae Yoon
- Department of Internal Medicine, Eunpyeong St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Kwang Hyung Chung
- Department of Internal Medicine, Uijeongbu Eulji Medical Center, Eulji University School of Medicine, Uijeongbu, Korea
| | - Jin Lee
- Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea
| | - Miyoung Choi
- National Evidence-Based Healthcare Collaborating Agency, Seoul, Korea
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Chon HK, Kim KH, Song TJ, Ahn DW, Lee ES, Lee YN, Lee YS, Jeon TJ, Park CH, Cho KB, Lee DW, Park JS, Yoon SB, Chung KH, Lee J, Choi M. Quality Indicators of Endoscopic Retrograde Cholangiopancreatography in Korea. Gut Liver 2024; 18:564-577. [PMID: 38462478 PMCID: PMC11249929 DOI: 10.5009/gnl230427] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/19/2023] [Accepted: 11/22/2023] [Indexed: 03/12/2024] Open
Abstract
Endoscopic retrograde cholangiopancreatography (ERCP) is a procedure that requires significant experiences and skills and has various procedure-related complications, some of which can be severe and even result in the death of patients. Expanding ERCP availability has the advantage of increasing accessibility for patients. However, ERCP poses a substantial risk if performed without proper quality management. ERCP quality management is essential for both ensuring safe and successful procedures and meeting the social demands for enhanced healthcare competitiveness and quality assurance. To address these concerns, the Korean Pancreatobiliary Association established a task force to develop ERCP quality indicators (QIs) tailored to the Korean medical environment. Key questions for five pre-procedure, three intra-procedure, and four post-procedure measures were formulated based on a literature search related to ERCP QIs and a comprehensive clinical review conducted by experts. The statements and recommendations regarding each QI item were selected through peer review. The developed ERCP QIs were reviewed by external experts based on the latest available evidence at the time of development. These domestically tailored ERCP QIs are expected to contribute considerably to improving ERCP quality in Korea.
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Affiliation(s)
- Hyung Ku Chon
- Department of Internal Medicine, Wonkwang University College of Medicine, Iksan, Korea
- Institute of Wonkwang Medical Science, Iksan, Korea
| | - Ki-Hyun Kim
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
| | - Tae Jun Song
- Department of Internal Medicine, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong-Won Ahn
- Department of Internal Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Korea
| | - Eaum Seok Lee
- Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea
| | - Yun Nah Lee
- Department of Internal Medicine, Soonchunhyang University School of Medicine, Bucheon, Korea
| | - Yoon Suk Lee
- Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea
| | - Tae Joo Jeon
- Department of Internal Medicine, Inje University Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea
| | - Chang Hwan Park
- Department of Internal Medicine, Chonnam National University Medical School, Gwangju, Korea
| | - Kwang Bum Cho
- Department of Internal Medicine, Keimyung University Dongsan Medical Center, Keimyung University School of Medicine, Daegu, Korea
| | - Dong Wook Lee
- Department of Internal Medicine, Kyungpook National University Chilgok Hospital, School of Medicine, Kyungpook National University, Daegu, Korea
| | - Jin-Seok Park
- Department of Internal Medicine, Inha University School of Medicine, Incheon, Korea
| | - Seung Bae Yoon
- Department of Internal Medicine, Eunpyeong St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea
| | - Kwang Hyun Chung
- Department of Internal Medicine, Uijeongbu Eulji Medical Center, Eulji University School of Medicine, Uijeongbu, Korea
| | - Jin Lee
- Division of Gastroenterology, Department of Internal Medicine, Hallym University Dongtan Sacred Heart Hospital, Hallym University College of Medicine, Hwaseong, Korea
| | - Miyoung Choi
- National Evidence-based Healthcare Collaborating Agency, Seoul, Korea
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Trauner M, Halilbasic E, Tatscher E, Fickert P. [Primary sclerosing cholangitis-Diagnosis and treatment 2024]. INNERE MEDIZIN (HEIDELBERG, GERMANY) 2024; 65:347-356. [PMID: 38498179 PMCID: PMC10959807 DOI: 10.1007/s00108-024-01697-0] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Subscribe] [Scholar Register] [Accepted: 03/05/2024] [Indexed: 03/20/2024]
Abstract
The etiology of primary sclerosing cholangitis (PSC) remains unclear, which explains in part the lack of a causal treatment. The differential diagnostic distinction from the even rarer immunoglobulin 4 (IgG4)-associated cholangitis (IAC) is becoming increasingly more successful. Advances in the understanding of different clinical courses, improvements in noninvasive diagnostics through modern magnetic resonance imaging (MRI) and the introduction of liver elastography have led to the development of improved prognostic models. The evidence for recommendations on medicinal (e.g., ursodeoxycholic acid) or endoscopic treatment (e.g., balloon dilatation and/or stent insertion) for PSC is still low. In contrast, the long-term results of liver transplantation in PSC patients are constantly improving. Due to the lack of highly sensitive and specific screening methods the early recognition of cholangiocellular carcinoma (CCC) as the most important complication is rarely successful. The continuous improvement of endoscopic retrograde cholangiopancreatography (ERCP) and direct cholangioscopy in combination with molecular biological and fluorescence in situ hybridization (FISH) analyses of bile duct tissue samples are promising for refined diagnostics. Due to the significantly increased risk of colorectal cancer, an annual colonoscopy is recommended in the presence of inflammatory bowel disease. Improvement of the early diagnostics of PSC and successful testing of new treatment strategies raise hope for a continuous improvement in the medical support of these complex patients.
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Affiliation(s)
- Michael Trauner
- Klinische Abteilung für Gastroenterologie und Hepatologie, Universitätsklinik für Innere Medizin III, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich.
| | - Emina Halilbasic
- Klinische Abteilung für Gastroenterologie und Hepatologie, Universitätsklinik für Innere Medizin III, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich
| | - Elisabeth Tatscher
- Klinische Abteilung für Gastroenterologie und Hepatologie, Universitätsklinik für Innere Medizin, Medizinische Universität Graz, Auenbruggerplatz 15, 8036, Graz, Österreich
| | - Peter Fickert
- Klinische Abteilung für Gastroenterologie und Hepatologie, Universitätsklinik für Innere Medizin, Medizinische Universität Graz, Auenbruggerplatz 15, 8036, Graz, Österreich.
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Tow CY, Chung E, Kaul B, Bhalla A, Fortune BE. Diagnostic Tests in Primary Sclerosing Cholangitis: Serology, Elastography, Imaging, and Histology. Clin Liver Dis 2024; 28:157-169. [PMID: 37945157 DOI: 10.1016/j.cld.2023.07.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/12/2023]
Abstract
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by progressive inflammation and fibrosis of the biliary tree leading to biliary strictures, cholangitis, and cirrhosis. Early in presentation, patients may have normal liver tests, though over time develop a cholestatic pattern of liver injury. Diagnosis is made radiographically with magnetic resonance or endoscopic cholangiography. While several autoantibodies are associated with PSC, none have proven to have adequate diagnostic utility. Liver biopsy is rarely recommended unless to evaluate for small-duct PSC or overlap syndrome. Elastography, in various forms, is an effective, non-invasive modality to evaluate liver fibrosis in PSC.
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Affiliation(s)
- Clara Y Tow
- Division of Hepatology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA; Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA
| | - Erica Chung
- Division of Gastroenterology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA
| | - Bindu Kaul
- Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA; Department of Radiology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA
| | - Amarpreet Bhalla
- Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA; Department of Pathology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA
| | - Brett E Fortune
- Division of Hepatology, Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA; Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA.
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Shah YR, Nombera-Aznaran N, Guevara-Lazo D, Calderon-Martinez E, Tiwari A, Kanumilli S, Shah P, Pinnam BSM, Ali H, Dahiya DS. Liver transplant in primary sclerosing cholangitis: Current trends and future directions. World J Hepatol 2023; 15:939-953. [PMID: 37701917 PMCID: PMC10494561 DOI: 10.4254/wjh.v15.i8.939] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/15/2023] [Revised: 07/23/2023] [Accepted: 08/11/2023] [Indexed: 08/22/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) is a chronic and progressive immune-mediated cholangiopathy causing biliary tree inflammation and scarring, leading to liver cirrhosis and end-stage liver disease. Diagnosis of PSC is challenging due to its nonspecific symptoms and overlap with other liver diseases. Despite the rising incidence of PSC, there is no proven medical therapy that can alter the natural history of the disease. While liver transplantation (LT) is the most effective approach for managing advanced liver disease caused by PSC, post-transplantation recurrence of PSC remains a challenge. Therefore, ongoing research aims to develop better therapies for PSC, and continued efforts are necessary to improve outcomes for patients with PSC. This article provides an overview of PSC's pathogenesis, clinical presentation, and management options, including LT trends and future aspects. It also highlights the need for improved therapeutic options and ethical considerations in providing equitable access to LT for patients with PSC. Additionally, the impact of liver transplant on the quality of life and psychological outcomes of patients with PSC is discussed. Ongoing research into PSC's pathogenesis and post-transplant recurrence is crucial for improved understanding of the disease and more effective treatment options.
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Affiliation(s)
- Yash R Shah
- Department of Internal Medicine, Trinity Health Oakland, Pontiac, MI 48341, United States
| | | | - David Guevara-Lazo
- Faculty of Medicine, Universidad Peruana Cayetano Heredia, Lima 15102, Peru
| | - Ernesto Calderon-Martinez
- Department of Internal Medicine, Universidad Nacional Autonoma de Mexico, Ciudad De Mexico 04510, Mexico
| | - Angad Tiwari
- Department of Internal Medicine, Maharani Laxmi Bai Medical College, Jhansi 284001, India
| | | | - Purva Shah
- Department of Postgraduate Education, Harvard Medical School, Boston, MA 02115, United States
| | - Bhanu Siva Mohan Pinnam
- Department of Internal Medicine, John H. Stroger Hospital of Cook County, Chicago, IL 60612, United States
| | - Hassam Ali
- Department of Internal Medicine, East Carolina University/Brody School of Medicine, Greenville, NC 27858, United States
| | - Dushyant Singh Dahiya
- Division of Gastroenterology, Hepatology and Motility, The University of Kansas School of Medicine, Kansas City, KS 66160, United States.
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10
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Wu CCH, Lim SJM, Khor CJL. Endoscopic retrograde cholangiopancreatography-related complications: risk stratification, prevention, and management. Clin Endosc 2023; 56:433-445. [PMID: 37460103 PMCID: PMC10393565 DOI: 10.5946/ce.2023.013] [Citation(s) in RCA: 10] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2022] [Accepted: 01/11/2023] [Indexed: 07/29/2023] Open
Abstract
Endoscopic retrograde cholangiopancreatography (ERCP) plays a crucial role in the management of pancreaticobiliary disorders. Although the ERCP technique has been refined over the past five decades, it remains one of the endoscopic procedures with the highest rate of complications. Risk factors for ERCP-related complications are broadly classified into patient-, procedure-, and operator-related risk factors. Although non-modifiable, patient-related risk factors allow for the closer monitoring and instatement of preventive measures. Post-ERCP pancreatitis is the most common complication of ERCP. Risk reduction strategies include intravenous hydration, rectal nonsteroidal anti-inflammatory drugs, and pancreatic stent placement in selected patients. Perforation is associated with significant morbidity and mortality, and prompt recognition and treatment of ERCP-related perforations are key to ensuring good clinical outcomes. Endoscopy plays an expanding role in the treatment of perforations. Specific management strategies depend on the location of the perforation and the patient's clinical status. The risk of post-ERCP bleeding can be attenuated by preprocedural optimization and adoption of intra-procedural techniques. Endoscopic measures are the mainstay of management for post-ERCP bleeding. Escalation to angioembolization or surgery may be required for refractory bleeding. Post-ERCP cholangitis can be reduced with antibiotic prophylaxis in high risk patients. Bile culture-directed therapy plays an important role in antimicrobial treatment.
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Affiliation(s)
- Clement Chun Ho Wu
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore
- Duke-NUS Medical School, Singapore
| | - Samuel Jun Ming Lim
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore
- Duke-NUS Medical School, Singapore
| | - Christopher Jen Lock Khor
- Department of Gastroenterology and Hepatology, Singapore General Hospital, Singapore
- Duke-NUS Medical School, Singapore
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Effenberger M, Al-Zoairy R, Gstir R, Graziadei I, Schwaighofer H, Tilg H, Zoller H. Transmission of oral microbiota to the biliary tract during endoscopic retrograde cholangiography. BMC Gastroenterol 2023; 23:103. [PMID: 37013522 PMCID: PMC10069052 DOI: 10.1186/s12876-023-02721-7] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/09/2022] [Accepted: 03/13/2023] [Indexed: 04/05/2023] Open
Abstract
BACKGROUND Endoscopic retrograde cholangiography (ERC) possesses a translocation risk of microbes to the biliary system. We studied bile contamination during ERC and its impact on patients' outcome in a real-life-situation. METHODS Ninety-nine ERCs were analyzed and microbial samples were taken from the throat before and from bile during ERC and from irrigation fluid of the duodenoscope before and after ERC. RESULTS 91.2% of cholangitis patients had detectable microbes in the bile (sensitivity 91%), but the same was true for 86.2% in the non-cholangitis group. Bacteroides fragilis (p=0.015) was significantly associated with cholangitis. In 41.7% of ERCs with contaminated endoscopes these microbes were found in the bile after the procedure. Analysis of duodenoscopes' irrigation liquid after ERC matched the microbial bile analysis of these patients in 78.8%. Identical microbial species were in throat and in bile samples of the same ERC in 33% of all cases and in 45% in the non-cholangitis group. Transmission of microbes to the biliary tract did not result in more frequent cholangitis, longer hospital stays, or worse outcome. CONCLUSIONS During ERC bile samples are regularly contaminated with microbes of the oral cavity but it did not affect clinical outcome.
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Affiliation(s)
- Maria Effenberger
- Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria
| | - Ramona Al-Zoairy
- Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria
| | - Ronald Gstir
- Division of Hygiene and Medical Microbiology, Medical University of Innsbruck, Innsbruck, Austria
| | - Ivo Graziadei
- Department of Internal Medicine, Academic Teaching Hospital Hall, Hall/Tirol, Austria
| | - Hubert Schwaighofer
- Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria
| | - Herbert Tilg
- Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria
| | - Heinz Zoller
- Department of Internal Medicine I, Gastroenterology, Hepatology, Endocrinology and Metabolism, Medical University of Innsbruck, Anichstrasse 35, Innsbruck, 6020, Austria.
- Christian Doppler Laboratory on Iron and Phosphate Biology, Medical University of Innsbruck, Innsbruck, Austria.
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12
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Bowlus CL, Arrivé L, Bergquist A, Deneau M, Forman L, Ilyas SI, Lunsford KE, Martinez M, Sapisochin G, Shroff R, Tabibian JH, Assis DN. AASLD practice guidance on primary sclerosing cholangitis and cholangiocarcinoma. Hepatology 2023; 77:659-702. [PMID: 36083140 DOI: 10.1002/hep.32771] [Citation(s) in RCA: 141] [Impact Index Per Article: 70.5] [Reference Citation Analysis] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2022] [Accepted: 07/26/2022] [Indexed: 01/28/2023]
Affiliation(s)
- Christopher L Bowlus
- Division of Gastroenterology , University of California Davis Health , Sacramento , California , USA
| | | | - Annika Bergquist
- Karolinska Institutet , Karolinska University Hospital , Stockholm , Sweden
| | - Mark Deneau
- University of Utah , Salt Lake City , Utah , USA
| | - Lisa Forman
- University of Colorado , Aurora , Colorado , USA
| | - Sumera I Ilyas
- Mayo Clinic College of Medicine and Science , Rochester , Minnesota , USA
| | - Keri E Lunsford
- Rutgers University-New Jersey Medical School , Newark , New Jersey , USA
| | - Mercedes Martinez
- Vagelos College of Physicians and Surgeons , Columbia University , New York , New York , USA
| | | | | | - James H Tabibian
- David Geffen School of Medicine at UCLA , Los Angeles , California , USA
| | - David N Assis
- Yale School of Medicine , New Haven , Connecticut , USA
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13
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Gustafsson A, Enochsson L, Tingstedt B, Olsson G. Antibiotic prophylaxis and its effect on postprocedural adverse events in endoscopic retrograde cholangiopancreatography for primary sclerosing cholangitis. JGH Open 2022; 7:24-29. [PMID: 36660048 PMCID: PMC9840194 DOI: 10.1002/jgh3.12846] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/23/2022] [Revised: 11/08/2022] [Accepted: 11/12/2022] [Indexed: 12/07/2022]
Abstract
Background and Aim Primary sclerosing cholangitis (PSC) is characterized by multiple strictures of the biliary tree. Patients with PSC frequently require repeated endoscopic retrograde cholangiopancreatography (ERCP) procedures. These procedures are encumbered by an increased incidence of infectious adverse events such as cholangitis. Evidence regarding whether antibiotic prophylaxis (AP) should be administered is sparse; however, prophylaxis is recommended. We aimed to determine whether AP affects the rate of postprocedural infectious and overall adverse events. Methods We conducted a retrospective cohort study and extracted all ERCP procedures with indicated PSC performed between 1 January 2006 and 31 December 2019, which were registered in the Swedish Registry for Gallstone Surgery and ERCP (GallRiks). The exclusion criteria were incomplete 30-day follow-up, non-index procedures, or ongoing antibiotics. The main outcomes were postprocedural infectious adverse events and overall adverse events at the 30-day follow-up. Results A total of 2144 procedures with indication of PSC were eligible for inclusion. AP was administered in 1407 (66%) of these procedures. Patients receiving AP were slightly younger (44 vs 46 years, P = 0.005) and had more comorbidities (ASA ≥3, 19.8% vs 13.6%; P < 0.001). Procedures with AP demonstrated an infectious adverse event rate of 3.3% compared to 4.5% for non-AP procedures (P = 0.19). Postprocedural infectious adverse events (odds ratio [OR] 0.76, 95% confidence interval [CI] 0.48-1.21) and overall adverse events (OR 0.79, 95% CI 0.60-1.04) did not differ between AP and non-AP. Conclusion Patients with PSC who undergo ERCP have the same frequency of adverse events regardless of whether AP was used.
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Affiliation(s)
- Arvid Gustafsson
- Department of Research and Development and Department of SurgeryCentral HospitalVäxjöSweden,Department of Clinical Sciences Lund, Surgery, Lund University and Department of SurgerySkåne University HospitalLundSweden
| | - Lars Enochsson
- Department of Surgical and Perioperative Sciences, SurgeryUmeå UniversityUmeåSweden
| | - Bobby Tingstedt
- Department of Clinical Sciences Lund, Surgery, Lund University and Department of SurgerySkåne University HospitalLundSweden
| | - Greger Olsson
- Department of Research and Development and Department of SurgeryCentral HospitalVäxjöSweden
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14
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Chazouilleres O, Beuers U, Bergquist A, Karlsen TH, Levy C, Samyn M, Schramm C, Trauner M. EASL Clinical Practice Guidelines on sclerosing cholangitis. J Hepatol 2022; 77:761-806. [PMID: 35738507 DOI: 10.1016/j.jhep.2022.05.011] [Citation(s) in RCA: 162] [Impact Index Per Article: 54.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/16/2022] [Accepted: 05/16/2022] [Indexed: 02/07/2023]
Abstract
Management of primary or secondary sclerosing cholangitis is challenging. These Clinical Practice Guidelines have been developed to provide practical guidance on debated topics including diagnostic methods, prognostic assessment, early detection of complications, optimal care pathways and therapeutic (pharmacological, endoscopic or surgical) options both in adults and children.
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15
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Natt N, Michael F, Michael H, Dubois S, Al Mazrou'i A. ERCP-Related Adverse Events in Primary Sclerosing Cholangitis: A Systematic Review and Meta-Analysis. Can J Gastroenterol Hepatol 2022; 2022:2372257. [PMID: 35910039 PMCID: PMC9334029 DOI: 10.1155/2022/2372257] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/21/2022] [Accepted: 06/23/2022] [Indexed: 11/18/2022] Open
Abstract
Background and Aims While endoscopic retrograde cholangiopancreatography (ERCP) is a valuable diagnostic and therapeutic tool in primary sclerosing cholangitis (PSC), there is conflicting data on associated adverse events. The aims of this systematic review and meta-analysis are to (1) compare ERCP-related adverse events in patients with and without PSC and (2) determine risk factors for ERCP-related adverse events in PSC. Methods Embase, PubMed, and CENTRAL were searched between January 1, 2000, and May 12, 2021. Eligible studies included adults with PSC undergoing ERCP and reported at least one ERCP-related adverse event (cholangitis, pancreatitis, bleeding, and perforation) or an associated risk factor. The risk of bias was assessed with the Newcastle-Ottawa scale and Cochrane Risk of Bias 2. Raw event rates were used to calculate odds ratios (ORs) and then pooled using random-effects models. Results Twenty studies met eligibility criteria, of which four were included in a meta-analysis comparing post-ERCP adverse events in patients with PSC (n = 715) to those without PSC (n = 9979). We found a significant threefold increase in the 30-day odds of cholangitis in PSC compared to those without (OR 3.263, 95% CI 1.076-9.896; p=0.037). However, there were no significant differences in post-ERCP pancreatitis (PEP), bleeding, or perforation. Due to limitations in primary data, only risk factors contributing to PEP could be analyzed. Accidental passage of the guidewire into the pancreatic duct (OR 7.444, 95% CI 3.328-16.651; p < 0.001; I 2 = 65.0%) and biliary sphincterotomy (OR 4.802, 95% CI 1.916-12.033; p=0.001; I 2 = 73.1%) were associated with higher odds of PEP in a second meta-analysis including five studies. Conclusions In the context of limited comparative data and heterogeneity, PSC patients undergoing ERCP have higher odds of cholangitis despite the majority receiving antibiotics. Additionally, accidental wire passage and biliary sphincterotomy increased the odds of PEP. Future studies on ERCP-related risks and preventive strategies are needed.
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Affiliation(s)
- Navneet Natt
- Northern Ontario School of Medicine University, Sudbury and Thunder Bay, Ontario, Canada
| | - Faith Michael
- Northern Ontario School of Medicine University, Sudbury and Thunder Bay, Ontario, Canada
| | | | - Sacha Dubois
- Northern Ontario School of Medicine University, Sudbury and Thunder Bay, Ontario, Canada
- Centre for Applied Health Research, St. Joseph's Care Group, Thunder Bay, Ontario, Canada
- School of Nursing, Faculty of Health and Behavioural Sciences, Lakehead University, Thunder Bay, Ontario, Canada
| | - Ahmed Al Mazrou'i
- Northern Ontario School of Medicine University, Sudbury and Thunder Bay, Ontario, Canada
- Department of Gastroenterology, Health Sciences North, Sudbury, Ontario, Canada
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16
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Bokemeyer A, Lenze F, Stoica V, Sensoy TS, Kabar I, Schmidt H, Ullerich H. Digital single-operator video cholangioscopy improves endoscopic management in patients with primary sclerosing cholangitis-a retrospective observational study. World J Gastroenterol 2022; 28:2201-2213. [PMID: 35721887 PMCID: PMC9157616 DOI: 10.3748/wjg.v28.i20.2201] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/02/2022] [Revised: 03/18/2022] [Accepted: 04/21/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Patients with primary sclerosing cholangitis (PSC) are at a high risk of developing cholestatic liver disease and biliary cancer, and endoscopy is crucial for the complex management of these patients.
AIM To clarify the utility of recently introduced digital single-operator video cholangioscopy (SOVC) for the endoscopic management of PSC patients.
METHODS In this observational study, all patients with a history of PSC and in whom digital SOVC (using the SpyGlass DS System) was performed between 2015 and 2019 were included and retrospectively analysed. Examinations were performed at a tertiary referral centre in Germany. In total, 46 SOVCs performed in 38 patients with a history of PSC were identified. The primary endpoint was the evaluation of dominant biliary strictures using digital SOVC, and the secondary endpoints were the performance of selective guidewire passage across biliary strictures and the diagnosis and treatment of biliary stone disease in PSC patients.
RESULTS The 22 of 38 patients had a dominant biliary stricture (57.9%). In 4 of these 22 patients, a cholangiocellular carcinoma was diagnosed within the stricture (18.2%). Diagnostic evaluation of dominant biliary strictures using optical signs showed a sensitivity of 75% and a specificity of 94.4% to detect malignant strictures, whereas SOVC-guided biopsies to gain tissue for histopathological analysis showed a sensitivity of 50% and a specificity of 100%. In 13% of examinations, SOVC was helpful for guidewire passage across biliary strictures that could not be passed by conventional methods (technical success rate 100%). Biliary stone disease was observed in 17.4% of examinations; of these, in 37.5% of examinations, biliary stones could only be visualized by SOVC and not by standard fluoroscopy. Biliary stone treatment was successful in all cases (100%); 25% required SOVC-assisted electrohydraulic lithotripsy. Complications, such as postinterventional cholangitis and pancreatitis, occurred in 13% of examinations; however, no procedure-associated mortality occurred.
CONCLUSION Digital SOVC is effective and safe for the endoscopic management of PSC patients and may be regularly considered an additive tool for the complex endoscopic management of these patients.
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Affiliation(s)
- Arne Bokemeyer
- Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany
- Department of Gastroenterology, Hepatology and Transplant Medicine, University Hospital Essen, Essen 45147, Germany
| | - Frank Lenze
- Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany
| | - Viorelia Stoica
- Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany
| | - Timur Selcuk Sensoy
- Department of Gastroenterology, Hepatology and Transplant Medicine, University Hospital Essen, Essen 45147, Germany
| | - Iyad Kabar
- Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany
| | - Hartmut Schmidt
- Department of Gastroenterology, Hepatology and Transplant Medicine, University Hospital Essen, Essen 45147, Germany
| | - Hansjoerg Ullerich
- Department of Medicine B (Gastroenterology, Hepatology, Endocrinology, Clinical Infectiology), University Hospital Muenster, Muenster 48149, Germany
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17
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Keihanian T, Barakat MT, Tejaswi S, Mishra R, Carlson CJ, Brandabur JJ, Girotra M. Role of Endoscopic Retrograde Cholangiopancreatography in the Diagnosis and Management of Cholestatic Liver Diseases. Clin Liver Dis 2022; 26:51-67. [PMID: 34802663 DOI: 10.1016/j.cld.2021.08.006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/31/2023]
Abstract
Cholestatic liver diseases (CLDs) occur as a result of bile duct injury, emanating into duct obstruction and bile stasis. Advances in radiological imaging in the last decade has replaced endoscopic retrograde cholangiopancreatography (ERCP) as the first diagnostic tool, except in certain groups of patients, such as those with ischemic cholangiopathy (IsC) or early stages of primary sclerosing cholangitis (PSC). ERCP provides an opportunity for targeted tissue acquisition for histopathological evaluation and carries a diverse therapeutic profile to restore bile flow. The aim of this review article is to appraise the diagnostic and therapeutic roles of ERCP in CLDs.
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Affiliation(s)
- Tara Keihanian
- Division of Gastroenterology, Baylor College of Medicine, 7200 Cambridge Street, Suite A8, Houston, TX 77030, USA
| | - Monique T Barakat
- Division of Gastroenterology, Stanford University School of Medicine, 300 Pasteur Drive # 5244, Stanford, CA 94304, USA
| | - Sooraj Tejaswi
- Division of Gastroenterology, Sutter Medical Group, 2068 John Jones Road, Davis, CA 95161, USA
| | - Rajnish Mishra
- Digestive Health Institute, Swedish Medical Center, 1221 Madison Street, Arnold Pavilion, Suite 1220, Seattle, WA 98104, USA
| | - Christopher J Carlson
- Digestive Health Institute, Swedish Medical Center, 1221 Madison Street, Arnold Pavilion, Suite 1220, Seattle, WA 98104, USA
| | - John J Brandabur
- Digestive Health Institute, Swedish Medical Center, 1221 Madison Street, Arnold Pavilion, Suite 1220, Seattle, WA 98104, USA
| | - Mohit Girotra
- Digestive Health Institute, Swedish Medical Center, 1221 Madison Street, Arnold Pavilion, Suite 1220, Seattle, WA 98104, USA.
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18
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Tejaswi S, Loehfelm TW, Olson KA. Cholangioscopy in primary sclerosing cholangitis: a case series of benign features. VideoGIE 2021; 6:431-437. [PMID: 34527845 PMCID: PMC8430051 DOI: 10.1016/j.vgie.2021.05.008] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/04/2022] Open
Abstract
Background and Aims Cholangioscopy is useful in establishing a visual diagnosis of cholangiocarcinoma (CCA), but this is harder to achieve in primary sclerosing cholangitis (PSC) because of the stricture-forming nature of the disease. Furthermore, it can be harder to differentiate malignant from benign features of the underlying inflammation. This case series demonstrates the varied features of nonmalignant inflammatory findings in PSC. Methods A single experienced endoscopist performed cholangioscopy for PSC cases referred for ERCP. Results Cholangioscopy in these 5 cases without CCA demonstrated the features of acute and chronic inflammation, acute inflammatory mass, dominant stricture, acute cholangitis in a duct with features of chronic inflammation with a large pigmented stone, and fibrostenotic disease. Cholangioscopic maneuvers such as advancement across strictures after balloon dilation, targeted mucosal biopsy, and electrohydraulic lithotripsy (EHL) of impacted stones are demonstrated. The relevant radiographic and histopathologic features of the disease accompany each case description. Regarding long-term prognosis, 1 case of acute inflammatory mass and a case of worsening liver function required a liver transplant evaluation, whereas the other 3 cases remain stable. Conclusions Cholangioscopic features of benign disease in PSC are varied. Knowledge of these features is essential in differentiating between benign and malignant findings. These features, combined with biopsy and cytology evaluation, can help in tailoring management in patients with benign PSC.
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Key Words
- ALP, alkaline phosphatase
- ALT, alanine aminotransferase
- AST, aspartate aminotransferase
- CBD, common bile duct
- CCA, cholangiocarcinoma
- CHD, common hepatic duct
- EHL, electrohydraulic lithotripsy
- FISH, fluorescent in situ hybridization
- LFTs, liver function tests
- MRI, magnetic resonance imaging
- PSC, primary sclerosing cholangitis
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Affiliation(s)
- Sooraj Tejaswi
- Division of Gastroenterology & Hepatology, University of California Davis School of Medicine, Sacramento, California
| | - Thomas W Loehfelm
- Department of Radiology, University of California Davis School of Medicine, Sacramento, California
| | - Kristin A Olson
- Department of Pathology and Laboratory Medicine, University of California Davis School of Medicine, Sacramento, California
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19
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Koskensalo V, Aronen P, Färkkilä M, Kylänpää L, Lindström O, Rainio M, Udd M, Jokelainen K, Tenca A. Use of thiopurines is not a risk factor for post-ERC pancreatitis in patients with primary sclerosing cholangitis. Dig Liver Dis 2021; 53:1020-1027. [PMID: 34116970 DOI: 10.1016/j.dld.2021.05.009] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2021] [Revised: 05/04/2021] [Accepted: 05/11/2021] [Indexed: 12/11/2022]
Abstract
INTRODUCTION Risk of post-ERC pancreatitis (PEP) in patients with primary sclerosing cholangitis (PSC) is 1-7.8%. PSC is often associated with inflammatory bowel disease and autoimmune hepatitis, which are usually treated with thiopurines. The role of thiopurines in PEP risk is still unclear. AIMS AND METHODS We evaluated the thiopurine use in PEP. The data of 354 PSC patients who underwent 985 ERCs between 2009 and 2018 were collected. 177 patients treated with thiopurines (study group, SG) and 177 controls (CG) were matched with a propensity score (PSM). Odds ratios (ORs) with 95% confidence interval (95% CI) were calculated. Multivariable logistic regression analysis and generalized linear mixed model were performed. The P-value <0.05 was significant. RESULTS In matched data, 472 ERCs were performed in SG and 513 in CG. Thiopurines were used in 373/472 (79.0%) ERCs in SG. The PEP rate was 5.3% in SG and 5.7% in CG (p = 0.889). Unintentional pancreatic duct cannulation (OR 1.28, 95%CI 1.07-1.51, p = 0.004), and periampullary diverticulum (OR 4.87, 95%CI 1.72-11.98, p = 0.001) increased the risk of PEP. CONCLUSION Prior or present thiopurine use did not increase the risk of PEP.
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Affiliation(s)
- Vilja Koskensalo
- Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland.
| | - Pasi Aronen
- Biostatistics Consulting Unit, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Martti Färkkilä
- Department of Gastroenterology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Leena Kylänpää
- Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Outi Lindström
- Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Mia Rainio
- Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Marianne Udd
- Department of Gastrointestinal Surgery, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Kalle Jokelainen
- Department of Gastroenterology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
| | - Andrea Tenca
- Department of Gastroenterology, Helsinki University Hospital and University of Helsinki, Helsinki, Finland
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20
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Waldthaler A, Schramm C, Bergquist A. Present and future role of endoscopic retrograde cholangiography in primary sclerosing cholangitis. Eur J Med Genet 2021; 64:104231. [PMID: 33905896 DOI: 10.1016/j.ejmg.2021.104231] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/06/2021] [Revised: 04/20/2021] [Accepted: 04/21/2021] [Indexed: 12/12/2022]
Abstract
Primary sclerosing cholangitis (PSC) is a rare, inflammatory cholestatic liver disease that causes biliary strictures which can lead to secondary complications. About 30-50% of PSC patients develop dominant strictures (DS) in the biliary tree, which are both the cause of jaundice and bacterial cholangitis as well as predilection spots for development of neoplastic development. Cancer is the most common cause of death in PSC. A central concern is to distinguish malignant from benign strictures, which eventually is done by invasive methods to obtain a brush cytology or biopsy sample, in most cases via endoscopic retrograde cholangiography-pancreatography (ERCP). Since medical therapies, like ursodesoxycholic acid or immunosuppressive drugs have no proven effect, therapeutic ERCP has become the primary management strategy to improve symptoms and in some patients may slow down disease progression. This article aims at outlining the current and emerging methods in ERCP in PSC patients.
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Affiliation(s)
- A Waldthaler
- Department of Medicine Huddinge, Functional Unit Endoscopy, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden; European Reference Network for Hepatological Diseases (ENR RARE-LIVER), Sweden.
| | - C Schramm
- Department of Medicine and Martin Zeitz Center for Rare Diseases, University Medical Center Hamburg-Eppendorf, Hamburg, Germany; European Reference Network for Hepatological Diseases (ENR RARE-LIVER), Sweden
| | - A Bergquist
- Department of Medicine Huddinge, Unit of Gastroenterology and Rheumatology, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden; European Reference Network for Hepatological Diseases (ENR RARE-LIVER), Sweden
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21
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Björnsson ES, Kalaitzakis E. Recent advances in the treatment of primary sclerosing cholangitis. Expert Rev Gastroenterol Hepatol 2021; 15:413-425. [PMID: 33283566 DOI: 10.1080/17474124.2021.1860751] [Citation(s) in RCA: 11] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Introduction: PSC is a rare liver disease that leads frequently to cirrhosis and need for liver transplantation. No medical treatment is of proven value. Liver transplantation is the only curative therapy available. There is a big medical need to find medical therapy that can alter the natural history of the disease.Areas covered: The authors highlight advances in PSC, based on recent literature retrieved from PubMed until September 2020 regarding both medical and endoscopic biliary therapy.Future possibilities for treatment of PSC are discussed.Expert opinion: Biliary endoscopy is the cornerstone in the treatment of dominant strictures. Single-user peroral cholangioscopy is an emerging modality. Balloon dilatation therapy is the treatment of choice of dominant strictures. The most promising medical therapies showing efficacy in phase II trials are nor-Ursodeoxycholic acid, obethicolic acid, the non-steroidal FXR agonist Cilofexor and Aldafermin, a synthetic analogue of FGF-19. Antibiotics, particularly vancomycin have shown potential benefits, particularly in children but phase III studies are lacking. In observational studies of effects of biological therapy in patients with IBD/PSC adalimumab was associated with reduction in ALP. Results of liver transplantation are favorable but recurrence can be of clinical relevance particularly in patients transplanted before the age of 40.
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Affiliation(s)
- Einar S Björnsson
- Department of Internal Medicine, Faculty of Medicine, University of Iceland, Division of Gastroenterology and Hepatology, Landspitali University Hospital of Iceland
| | - Evangelos Kalaitzakis
- Department of Internal Medicine, University Hospital Heraklion, Faculty of Medicine, University of Crete, Rethymno, Greece
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22
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Abstract
Background and Aims Prompt and accurate differentiation of benign and malignant strictures in primary sclerosing cholangitis (PSC) is crucial. ERCP with brush cytology, the most common modality to achieve this, is hindered by a low diagnostic yield. Cholangioscopy can overcome this limitation by establishing a visual diagnosis based on the characteristic morphologic features of cholangiocarcinoma (CCA) and can aid in targeted biopsies of suspicious lesions. However, its role in PSC remains unclear. This case series demonstrates the performance of the latest generation of single-operator cholangioscope for this indication. Methods A single experienced endoscopist performed cholangioscopy for PSC cases referred for ERCP. Results Cholangioscopies of patients 1 to 3 demonstrate the features of extrahepatic duct dominant strictures (DS) and the cholangioscopic maneuvers undertaken in these cases, including advancement across the DS after balloon dilation, biopsy of the DS, and electrohydraulic lithotripsy of impacted stones. Cholangioscopies of patients 4 to 6 demonstrate the varied features of CCA ranging from focal stricture with tumor vessels, papillary frond-like projections, and features of an intraductal papillary biliary neoplasm. Also shown are the radiographic and histopathologic features of the disease. Conclusions Cholangioscopy allowed us to identify morphologic features of both malignancy and benign disease in PSC in the setting of extrahepatic duct strictures, and we were able to obtain adequate targeted tissue samples for histopathologic confirmation.
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Bhat P, Aabakken L. Role of Endoscopy in Primary Sclerosing Cholangitis. Clin Endosc 2020; 54:193-201. [PMID: 32380796 PMCID: PMC8039754 DOI: 10.5946/ce.2020.019-iden] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/07/2020] [Accepted: 03/23/2020] [Indexed: 11/24/2022] Open
Abstract
Primary sclerosing cholangitis (PSC) is a progressive disease of the bile ducts that usually results in chronic liver disease often requiring liver transplantation. Endoscopy remains crucial to the care of these patients, although magnetic resonance cholangiopancreatography has replaced endoscopic retrograde cholangiopancreatography (ERCP) as the primary imaging modality for diagnosis. For detection of dysplasia or cholangiocarcinoma, ERCP with intraductal sampling remains compulsory. Moreover, dominant strictures play an important part in the disease development, and management by balloon dilatation or stenting could contribute to long-term prognosis. In addition, endoscopy offers management for adverse events such as bile leaks and anastomotic strictures after liver transplantation. Finally, the special phenotype of inflammatory bowel disease associated with PSC as well as the frequent occurrence of portal hypertension mandates close follow-up with colonoscopy and upper endoscopy. With the emergence of novel techniques, the endoscopist remains a key member of the multidisciplinary team caring for PSC patients.
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Affiliation(s)
- Purnima Bhat
- Gastroenterology and Hepatology Unit, Canberra Hospital, Canberra, Australia.,College of Health and Medicine, Australian National University, Canberra, Australia
| | - Lars Aabakken
- Department of Transplantation Medicine, Oslo University Hospital, Oslo, Norway.,Faculty of Medicine, University of Oslo, Oslo, Norway
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24
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Koskensalo V, Tenca A, Udd M, Lindström O, Rainio M, Jokelainen K, Kylänpää L, Färkkilä M. Diclofenac does not reduce the risk of acute pancreatitis in patients with primary sclerosing cholangitis after endoscopic retrograde cholangiography. United European Gastroenterol J 2020; 8:462-471. [PMID: 32213036 DOI: 10.1177/2050640620912608] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND The European Society of Gastrointestinal Endoscopy recommends rectal indomethacin or diclofenac before endoscopic retrograde cholangiopancreatography (ERCP) to prevent post-ERCP pancreatitis. However, data on the prophylactic effect in patients with primary sclerosing cholangitis (PSC) are lacking. METHODS This was a retrospective case-control study. In 2009-2018, a total of 2000 ERCPs were performed in 931 patients with PSC. Case procedures (N = 1000 after November 2013) were performed after administration of rectal diclofenac. Control procedures (N = 1000 before November 2013) were performed with the same indication but without diclofenac. Acute post-ERCP pancreatitis and other ERCP-related adverse events (AEs) were evaluated. RESULTS Post-ERCP pancreatitis developed in 49 (4.9%) procedures in the diclofenac group and 62 (6.2%) procedures in the control group (p = 0.241). No difference existed between the groups in terms of the severity of pancreatitis or any other acute AEs. The risk of pancreatitis was elevated in patients with native papilla: 11.4% in the diclofenac group and 8.7% in the control group (p = 0.294). In adjusted logistic regression, diclofenac did not reduce the risk of pancreatitis (odds ratio (OR) = 1.074, 95% confidence interval 0.708-1.629, p = 0.737). However, in generalised estimation equations with the advanced model, diclofenac seemed to diminish the risk of pancreatitis (OR = 0.503) but not significantly (p = 0.110). CONCLUSION In this large patient cohort in a low-risk unit, diclofenac does not seem to reduce the risk of post-ERCP pancreatitis in patients with PSC. The trend in the pancreatitis rate after ERCP is decreasing. The evaluation of the benefits of diclofenac among PSC patients warrants a randomised controlled study targeted to high-risk patients and procedures.
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Affiliation(s)
- Vilja Koskensalo
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterological Surgery, Helsinki University Hospital, Helsinki, Finland
| | - Andrea Tenca
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterology, Helsinki University Hospital, Helsinki, Finland
| | - Marianne Udd
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterological Surgery, Helsinki University Hospital, Helsinki, Finland
| | - Outi Lindström
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterological Surgery, Helsinki University Hospital, Helsinki, Finland
| | - Mia Rainio
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterological Surgery, Helsinki University Hospital, Helsinki, Finland
| | - Kalle Jokelainen
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterology, Helsinki University Hospital, Helsinki, Finland
| | - Leena Kylänpää
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterological Surgery, Helsinki University Hospital, Helsinki, Finland
| | - Martti Färkkilä
- Faculty of Medicine, University of Helsinki, Helsinki, Finland.,Abdominal Centre, Gastroenterology, Helsinki University Hospital, Helsinki, Finland
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25
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Rupp C, Hippchen T, Bruckner T, Klöters-Plachky P, Schaible A, Koschny R, Stiehl A, Gotthardt DN, Sauer P. Effect of scheduled endoscopic dilatation of dominant strictures on outcome in patients with primary sclerosing cholangitis. Gut 2019; 68:2170-2178. [PMID: 30910856 PMCID: PMC6872453 DOI: 10.1136/gutjnl-2018-316801] [Citation(s) in RCA: 35] [Impact Index Per Article: 5.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/14/2018] [Revised: 02/16/2019] [Accepted: 03/08/2019] [Indexed: 12/12/2022]
Abstract
OBJECTIVE Scheduled endoscopic dilatation of dominant strictures (DS) in primary sclerosing cholangitis (PSC) might improve outcome relative to endoscopic treatment on demand, but evidence is limited. Since randomisation is difficult in clinical practice, we present a large retrospective study comparing scheduled versus on-demand endoscopic retrograde cholangiopancreatography (ERCP) based on patient preferences. DESIGN Between 1987 and 2017, all new patients with PSC had been offered scheduled ERCP with dilatation of a DS if diagnosed; the latter was repeated at defined intervals until morphological resolution, independent of clinical symptoms (treatment group). Patients who refused participation were clinically evaluated annually and received endoscopic treatment only on demand (control group). The primary clinical endpoint was transplantation-free survival. Secondary outcomes were overall survival, bacterial cholangitis episodes, hepatic decompensation of liver cirrhosis and endoscopy-related adverse events. RESULTS The final study included 286 patients, 133 (46.5%) receiving scheduled ERCP and 153 (53.5%) receiving on-demand ERCP. After a mean follow-up of 9.9 years, the rate of transplantation-free survival was higher in patients receiving scheduled ERCP (51% vs 29.3%; p<0.001), as was transplantation-free survival time (median: 17.9 vs 15.2 years; log-rank: p=0.008). However, the benefit of scheduled ERCP was significant only in patients with the initial (17.1%) or later (45.5%) diagnosis of a DS (17.8 vs 11.1 years; log-rank: p<0.001). IBD (p=0.03), DS (p=0.006), higher Mayo Risk Score (p=0.02) and non-adherence to scheduled endoscopy (p=0.005) were independently associated with transplantation-free survival. CONCLUSION In our large retrospective study, regular ERCP with endoscopic balloon dilatation significantly benefits patients with PSC with DS, diagnosed both at initial presentation and during surveillance, even if asymptomatic. Further studies have to find out how to best identify stricture patients non-invasively.
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Affiliation(s)
- Christian Rupp
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany,Department of Internal Medicine IV, Interdisciplinary Endoscopy Center, University Hospital Heidelberg, Heidelberg, Germany
| | - Theresa Hippchen
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany
| | - Thomas Bruckner
- Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany
| | - Petra Klöters-Plachky
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany
| | - Anja Schaible
- Department of General, Visceral and Transplantation Surgery, Interdisciplinary Endoscopy Center, University Hospital of Heidelberg, Heidelberg, Germany
| | - Ronald Koschny
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany,Department of Internal Medicine IV, Interdisciplinary Endoscopy Center, University Hospital Heidelberg, Heidelberg, Germany
| | - Adolf Stiehl
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany
| | - Daniel Nils Gotthardt
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany
| | - Peter Sauer
- Department of Internal Medicine IV, University Hospital of Heidelberg, Heidelberg, Germany,Department of Internal Medicine IV, Interdisciplinary Endoscopy Center, University Hospital Heidelberg, Heidelberg, Germany
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26
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Chapman MH, Thorburn D, Hirschfield GM, Webster GGJ, Rushbrook SM, Alexander G, Collier J, Dyson JK, Jones DE, Patanwala I, Thain C, Walmsley M, Pereira SP. British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis. Gut 2019; 68:1356-1378. [PMID: 31154395 PMCID: PMC6691863 DOI: 10.1136/gutjnl-2018-317993] [Citation(s) in RCA: 179] [Impact Index Per Article: 29.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/25/2018] [Revised: 02/21/2019] [Accepted: 03/24/2019] [Indexed: 12/11/2022]
Abstract
These guidelines on the management of primary sclerosing cholangitis (PSC) were commissioned by the British Society of Gastroenterology liver section. The guideline writing committee included medical representatives from hepatology and gastroenterology groups as well as patient representatives from PSC Support. The guidelines aim to support general physicians, gastroenterologists and surgeons in managing adults with PSC or those presenting with similar cholangiopathies which may mimic PSC, such as IgG4 sclerosing cholangitis. It also acts as a reference for patients with PSC to help them understand their own management. Quality of evidence is presented using the AGREE II format. Guidance is meant to be used as a reference rather than for rigid protocol-based care as we understand that management of patients often requires individual patient-centred considerations.
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Affiliation(s)
- Michael Huw Chapman
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Liver Unit, Royal Free London NHS Foundation Trust, London, UK
| | | | - Gideon M Hirschfield
- Toronto Centre for Liver Disease, University Health Network and University of Toronto, Toronto, Canada
| | | | - Simon M Rushbrook
- Department of Hepatology, Norfolk and Norwich University Hospitals NHS Trust, Norwich, UK
| | | | | | - Jessica K Dyson
- Hepatology, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - David Ej Jones
- Institute of Cellular Medicine, Newcastle University, Newcastle, UK
| | - Imran Patanwala
- Gastroenterology, Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK
- University of Liverpool, Liverpool, UK
| | | | | | - Stephen P Pereira
- GI Division, UCL Hospitals NHS Foundation Trust, London, UK
- Institute for Liver & Digestive Health, University College London, London, UK
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27
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Fung BM, Tabibian JH. Biliary endoscopy in the management of primary sclerosing cholangitis and its complications. LIVER RESEARCH (BEIJING, CHINA) 2019; 3:106-117. [PMID: 31341699 PMCID: PMC6656407 DOI: 10.1016/j.livres.2019.03.004] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Primary sclerosing cholangitis (PSC) is a chronic, idiopathic, cholestatic liver disease characterized by inflammation and fibrosis of the intrahepatic and/or extrahepatic bile ducts. It can affect individuals of all age groups and gender, has no established pharmacotherapy, and is associated with a variety of neoplastic (e.g. cholangiocarcinoma) and non-neoplastic (e.g. dominant strictures) hepatobiliary complications. Given these considerations, endoscopy plays a major role in the care of patients with PSC. In this review, we discuss and provide updates regarding endoscopic considerations in the management of hepatobiliary manifestations and complications of PSC. Where evidence is limited, we suggest pragmatic approaches based on currently available data and expert opinion.
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Affiliation(s)
- Brian M. Fung
- University of California Los Angeles-Olive View Internal Medicine Residency Program, Sylmar, CA, USA
| | - James H. Tabibian
- Division of Gastroenterology, Department of Medicine, Olive View-University of California Los Angeles Medical Center, Sylmar, CA, USA
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28
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Primary Sclerosing Cholangitis: A Concise Review of Diagnosis and Management. Dig Dis Sci 2019; 64:632-642. [PMID: 30725292 DOI: 10.1007/s10620-019-05484-y] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/22/2018] [Accepted: 01/19/2019] [Indexed: 02/08/2023]
Abstract
Primary sclerosing cholangitis is a rare, chronic cholestatic liver disease characterized by progressive idiopathic stricturing of the biliary system, typically leading to cirrhosis, end-stage liver disease, and colonic or hepatobiliary malignancy. Its presentation is often that of asymptomatic alkaline phosphatase elevation. When symptoms are present, they typically include fatigue, pruritus, or jaundice. The diagnosis can be confirmed via cholangiography, either magnetic resonance cholangiography (MRCP) or endoscopic retrograde cholangiography if the former is inconclusive. The clinical course is marked by progressive liver disease leading to cirrhosis with its attendant complications of portal hypertension, often including recurrent episodes of cholangitis. Greater elevation in alkaline phosphatase or liver stiffness is associated with worse clinical outcomes. Management includes endoscopic treatment of symptomatic biliary strictures and evaluation of dominant strictures as no adequate medical treatment is available. Multiple medical therapies are under evaluation. Ultimately, liver transplantation may be necessary for management of decompensated cirrhosis or disabling symptoms. There is also a markedly increased risk of cancer, notably including cholangiocarcinoma and gallbladder and colorectal cancers (particularly in patients with colitis). Cancer screening can be done with semi-annual liver imaging (MRCP or ultrasound) and colonoscopy every 1-2 years in those with colitis.
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29
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Marya NB, Tabibian JH. Role of endoscopy in the management of primary sclerosing cholangitis. World J Gastrointest Endosc 2019; 11:84-94. [PMID: 30788027 PMCID: PMC6379747 DOI: 10.4253/wjge.v11.i2.84] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/28/2018] [Revised: 01/15/2019] [Accepted: 01/30/2019] [Indexed: 02/06/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) is a rare but prominent fibroinflammatory cholangiopathy which can affect individuals of essentially any age. It carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent risk for biliary tract and other complications, and the paucity of effective pharmacotherapies, endoscopy plays a major role in the care of many patients with this disorder. In this review, we discuss the endoscopic management of PSC, including established and evolving approaches to the diagnosis and treatment of its benign as well as malignant sequelae. Owing to the rarity of PSC and dearth of high-quality evidence, we propose pragmatic approaches based on both currently available data and expert opinion.
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Affiliation(s)
- Neil Bharat Marya
- Vatche and Tamar Manoukian Division of Digestive Diseases, UCLA Gastroenterology Fellowship Training Program, Los Angeles, CA 90095, United States
| | - James H Tabibian
- Division of Gastroenterology, Department of Medicine, Olive View-UCLA Medical Center, Sylmar, CA 91342, United States
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30
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Ahmad J. Metal, magnet or transplant: options in primary sclerosing cholangitis with stricture. Hepatol Int 2018; 12:510-519. [PMID: 30430358 DOI: 10.1007/s12072-018-9906-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2018] [Accepted: 10/20/2018] [Indexed: 02/06/2023]
Abstract
Primary sclerosing cholangitis (PSC) is a chronic inflammatory disease of the biliary tree of unknown etiology leading to stricturing and dilation. There is currently no effective medical therapy for PSC and liver transplantation (LT) remains the ultimate treatment for severe disease defined as repeated episodes of cholangitis, decompensated biliary cirrhosis or in exceptional cases, cholangiocarcinoma (CCA). Patients often present with a "dominant" stricture and the therapeutic endoscopist plays an important role in management to improve biliary patency using a variety of techniques that involve sampling, balloon dilation and temporary stenting. Newer modalities such as self-expanding metal stents or magnetic compression anastomosis that have been used in other diseases may have a role to play in PSC but should remain investigational. Liver transplantation for PSC is curative in most cases but the optimal timing remains unclear. The lifetime risk of CCA is 10-15% in PSC patients and LT is often not possible at the time of diagnosis. Multiple studies have tried to identify risk factors and to diagnose CCA at an early stage when surgical resection may be possible or LT can be performed. However, deceased donor organs for LT remain in short supply throughout the world so even identifying PSC patients with CCA at an early stage may not be beneficial unless a live donor organ is available.
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Affiliation(s)
- Jawad Ahmad
- Division of Liver Diseases, Icahn School of Medicine at Mount Sinai, New York, NY, 10029, USA.
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31
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Ponsioen CY, Arnelo U, Bergquist A, Rauws EA, Paulsen V, Cantú P, Parzanese I, De Vries EM, van Munster KN, Said K, Chazouillères O, Desaint B, Kemgang A, Färkkilä M, Van der Merwe S, Van Steenbergen W, Marschall HU, Stotzer PO, Thorburn D, Pereira SP, Aabakken L. No Superiority of Stents vs Balloon Dilatation for Dominant Strictures in Patients With Primary Sclerosing Cholangitis. Gastroenterology 2018; 155:752-759.e5. [PMID: 29803836 DOI: 10.1053/j.gastro.2018.05.034] [Citation(s) in RCA: 54] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/14/2018] [Revised: 05/01/2018] [Accepted: 05/08/2018] [Indexed: 02/07/2023]
Abstract
BACKGROUND & AIMS Dominant strictures occur in approximately 50% of patients with primary sclerosing cholangitis (PSC). Short-term stents have been reported to produce longer resolution of dominant strictures than single-balloon dilatation. We performed a prospective study to compare the efficacy and safety of balloon dilatation vs short-term stents in patients with non-end-stage PSC. METHODS We performed an open-label trial of patients with PSC undergoing therapeutic endoscopic retrograde cholangiopancreatography (ERCP) at 9 tertiary-care centers in Europe, from July 2011 through April 2016. Patients found to have a dominant stricture during ERCP were randomly assigned to groups that underwent balloon dilatation (n = 31) or stent placement for a maximum of 2 weeks (n = 34); patients were followed for 24 months. The primary outcome was the cumulative recurrence-free patency of the primary dominant strictures. RESULTS Study recruitment was terminated after a planned interim analysis because of futility and differences in treatment-related serious adverse events (SAEs) between groups. The cumulative recurrence-free rate did not differ significantly between groups (0.34 for the stent group and 0.30 for the balloon dilatation group at 24 months; P = 1.0). Most patients in both groups had reductions in symptoms at 3 months after the procedure. There were 17 treatment-related SAEs: post-ERCP pancreatitis in 9 patients and bacterial cholangitis in 4 patients. SAEs occurred in 15 patients in the stent group (45%) and in only 2 patients in the balloon dilatation group (6.7%) (odds ratio, 11.7; 95% confidence interval, 2.4-57.2; P = .001). CONCLUSIONS In a multicenter randomized trial of patients with PSC and a dominant stricture, short-term stents were not superior to balloon dilatation and were associated with a significantly higher occurrence of treatment-related SAEs. Balloon dilatation should be the initial treatment of choice for dominant strictures in patients with PSC. This may be particularly relevant to patients with an intact papilla. ClinicalTrials.gov no. NCT01398917.
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Affiliation(s)
- Cyriel Y Ponsioen
- Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands.
| | - Urban Arnelo
- Department of Gastroenterology & Hepatology, Karolinska University Hospital, Huddinge, Karolinska Institutet, Sweden
| | - Annika Bergquist
- Department of Gastroenterology & Hepatology, Karolinska University Hospital, Huddinge, Karolinska Institutet, Sweden
| | - Erik A Rauws
- Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands
| | - Vemund Paulsen
- Department of Gastroenterology & Hepatology, Rikshospitalet, Oslo, Norway
| | - Paolo Cantú
- Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
| | - Ilaria Parzanese
- Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy
| | - Elisabeth M De Vries
- Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands
| | - Kim N van Munster
- Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, the Netherlands
| | - Karouk Said
- Department of Gastroenterology & Hepatology, Karolinska University Hospital, Huddinge, Karolinska Institutet, Sweden
| | | | - Benoit Desaint
- Department of Hepatology, Hôpital Saint Antoine, Paris, France
| | - Astrid Kemgang
- Department of Hepatology, Hôpital Saint Antoine, Paris, France
| | - Martti Färkkilä
- Department of Gastroenterology & Hepatology, Helsinki University Hospital, Helsinki, Finland
| | - Schalk Van der Merwe
- Department of Gastroenterology & Hepatology, Universiteitsziekenhuis Leuven, Leuven, Belgium
| | - Werner Van Steenbergen
- Department of Gastroenterology & Hepatology, Universiteitsziekenhuis Leuven, Leuven, Belgium
| | | | - Per-Ove Stotzer
- Department of Hepatology, Sahlgrenska University, Gothenburg, Sweden
| | - Douglas Thorburn
- Institute of Liver & Digestive Health, University College London and Sheila Sherlock Liver Centre, Royal Free Hospital, London, United Kingdom
| | - Stephen P Pereira
- Institute of Liver & Digestive Health, University College London and Sheila Sherlock Liver Centre, Royal Free Hospital, London, United Kingdom
| | - Lars Aabakken
- Department of Gastroenterology & Hepatology, Rikshospitalet, Oslo, Norway
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32
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Isayama H, Tazuma S, Kokudo N, Tanaka A, Tsuyuguchi T, Nakazawa T, Notohara K, Mizuno S, Akamatsu N, Serikawa M, Naitoh I, Hirooka Y, Wakai T, Itoi T, Ebata T, Okaniwa S, Kamisawa T, Kawashima H, Kanno A, Kubota K, Tabata M, Unno M, Takikawa H. Clinical guidelines for primary sclerosing cholangitis 2017. J Gastroenterol 2018; 53:1006-1034. [PMID: 29951926 PMCID: PMC8930933 DOI: 10.1007/s00535-018-1484-9] [Citation(s) in RCA: 36] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2018] [Accepted: 06/11/2018] [Indexed: 02/04/2023]
Abstract
BACKGROUND Primary sclerosing cholangitis (PSC) is relatively rare disease and pathogenesis and methods of treatments were still not established. Then, we had conducted the making clinical guidelines to manage patients with PSC based on the literature review and expert opinions. These clinical guidelines were made for the medical doctors on the management of PSC, except child case of PSC. METHODS We had employed modified Delphi method. The production committee decided guidelines, strength of recommendations and evidence level after reviewed literatures systematically, and The Expert panel evaluated those. The Scientific Committee of the Japan Biliary Association (JBA) evaluated revised guidelines, and the Public comments were collected on web site of JBA. RESULTS We had made 16 guidelines about epidemiology/pathophysiology, diagnostics, therapy and prognosis. Also, we had made both diagnostic and therapeutic flow chart. CONCLUSIONS We hope that these guidelines will contribute to the improvement and development of the medical care of PSC.
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Affiliation(s)
- Hiroyuki Isayama
- Department of Gastroenterology, Graduate School of Medicine, Juntendo University, Tokyo, Japan
| | - Susumu Tazuma
- Department of General Internal Medicine, Graduate School of Biomedical and Health Sciences, Hiroshima University Hospital, 1-2-3, Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
| | - Norihiro Kokudo
- Department of Surgery, National Center for Global Health and Medicine, Tokyo, Japan
| | - Atsushi Tanaka
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
| | - Toshio Tsuyuguchi
- Department of Medicine and Gastroenterology, Chiba University, Chiba, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Japanese Red Cross Nagoya Daini Hospital, Nagoya, Japan
| | - Kenji Notohara
- Department of Anatomic Pathology, Kurashiki Central Hospital, Kurashiki, Japan
| | - Suguru Mizuno
- Department of Gastroenterology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Nobuhisa Akamatsu
- Artificial Organ and Transplantation Division, Department of Surgery, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masahiro Serikawa
- Department of Gastroenterology and Metabolism, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Hiroshima, Japan
| | - Itaru Naitoh
- Department of Gastroenterology and Metabolism, Graduate School of Medical Sciences, Nagoya City University, Nagoya, Japan
| | - Yoshiki Hirooka
- Department of Endoscopy, Nagoya University Hospital, Nagoya, Japan
| | - Toshifumi Wakai
- Division of Digestive and General Surgery, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
| | - Takao Itoi
- Department of Gastroenterology and Hepatology, Tokyo Medical University, Tokyo, Japan
| | - Tomoki Ebata
- Division of Surgical Oncology, Department of Surgery, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Shinji Okaniwa
- Department of Gastroenterology, Iida Municipal Hospital, Nagano, Japan
| | - Terumi Kamisawa
- Department of Internal Medicine, Tokyo Komagome Metropolitan Hospital, Tokyo, Japan
| | - Hiroki Kawashima
- Department of Gastroenterology, Nagoya University Graduate School of Medicine, Nagoya, Japan
| | - Atsushi Kanno
- Division of Gastroenterology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
| | - Keiichi Kubota
- Second Department of Surgery, Dokkyo Medical University, Tochigi, Japan
| | - Masami Tabata
- Department of Surgery, Matsusaka Central General Hospital, Matsusaka, Mie, Japan
| | - Michiaki Unno
- Department of Surgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
| | - Hajime Takikawa
- Department of Medicine, Teikyo University School of Medicine, Tokyo, Japan
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33
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Peiseler M, Reiners D, Pinnschmidt HO, Sebode M, Jung F, Hartl J, Zenouzi R, Ehlken H, Groth S, Schachschal G, Rösch T, Weiler-Normann C, Lohse AW, Schramm C. Risk of endoscopic biliary interventions in primary sclerosing cholangitis is similar between patients with and without cirrhosis. PLoS One 2018; 13:e0202686. [PMID: 30125307 PMCID: PMC6101401 DOI: 10.1371/journal.pone.0202686] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2018] [Accepted: 08/06/2018] [Indexed: 12/12/2022] Open
Abstract
Background Endoscopic retrograde cholangiography (ERC) is a mainstay of therapy in patients with primary sclerosing cholangitis (PSC) and obstructive cholestasis. Patients with liver cirrhosis have an increased risk of surgical complications and are more susceptible to infections. Since PSC often progresses to cirrhosis, we aimed to assess whether ERC is associated with increased risk of complications in patients with PSC and cirrhosis. Methods Out of 383 patients with PSC, 208 patients received endoscopic treatment between 2009–2017. Seventy patients had cirrhosis when ERC was performed and 138 patients had no signs of cirrhosis. Overall, 663 ERC procedures were analysed, with 250 ERC in patients with cirrhosis and 413 ERC in patients without cirrhosis. Data were analysed retrospectively from a prospectively acquired database using repeated measures logistic regression. Results Overall, 40 procedure-related complications were documented in 663 ERC interventions (6%). The rate of complications was similar between patients with and without cirrhosis (4.4% vs. 7.0%). First-time ERC was associated with a higher risk of complications (17.5% vs. 4.9%). Biliary sphincterotomy, stent placement and female sex, but not presence of liver cirrhosis, were identified as risk factors for overall complications in multivariate analysis. Patients without cirrhosis showed a significant decline of ALP and bilirubin levels after the first two interventions. In contrast, in patients with cirrhosis, ALP and bilirubin levels did not significantly decline after ERC. Conclusions In patients with PSC, cirrhosis was not a risk factor for post-ERC complications. Therefore, cirrhosis should not preclude endoscopic intervention in patients with clear clinical indication.
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Affiliation(s)
- Moritz Peiseler
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
- * E-mail: (MP); (CS)
| | - David Reiners
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Hans O. Pinnschmidt
- Department of Medical Biometry and Epidemiology, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Marcial Sebode
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Franziska Jung
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Johannes Hartl
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Roman Zenouzi
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Hanno Ehlken
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
- Department of Interdisciplinary Endoscopy, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Stefan Groth
- Department of Interdisciplinary Endoscopy, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Guido Schachschal
- Department of Interdisciplinary Endoscopy, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Thomas Rösch
- Department of Interdisciplinary Endoscopy, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Christina Weiler-Normann
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
- Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Ansgar W. Lohse
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
| | - Christoph Schramm
- I. Department of Medicine, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
- Martin Zeitz Centre for Rare Diseases, University Medical Centre Hamburg-Eppendorf, Hamburg, Germany
- * E-mail: (MP); (CS)
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Tabibian JH, Baron TH. Endoscopic management of primary sclerosing cholangitis. Expert Rev Gastroenterol Hepatol 2018; 12:693-703. [PMID: 29883229 DOI: 10.1080/17474124.2018.1483719] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2018] [Accepted: 05/30/2018] [Indexed: 12/14/2022]
Abstract
Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by intra- and/or extra-hepatic bile duct inflammation and fibro-obliteration, which ultimately leads to biliary cirrhosis and related sequelae, including development of hepatobiliary and colorectal carcinomata. PSC can be diagnosed at essentially any age and carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent association with both neoplastic and non-neoplastic biliary tract complications, and the lack of effective pharmacotherapies, alimentary and biliary tract endoscopy plays a major role in the care of patients with PSC. Areas covered: Here, we provide a narrative review on endoscopic management of PSC, including established and evolving applications to the diagnosis and treatment of both its benign and malignant complications. Expert commentary: Due to the rarity of PSC and the considerable patient-years required to rigorously study major endpoints, there remains a paucity of high-quality evidence regarding its management. As the advanced endoscopic repertoire expands, so has the interest in developing best practices in PSC, which we discuss herein.
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Affiliation(s)
- James H Tabibian
- a Division of Gastroenterology, Department of Medicine , Olive View-UCLA Medical Center , Sylmar , CA , USA
| | - Todd H Baron
- b Division of Gastroenterology and Hepatology , University of North Carolina at Chapel Hill , Chapel Hill , NC , USA
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Mohammad Alizadeh AH. Cholangitis: Diagnosis, Treatment and Prognosis. J Clin Transl Hepatol 2017; 5:404-413. [PMID: 29226107 PMCID: PMC5719198 DOI: 10.14218/jcth.2017.00028] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/28/2017] [Revised: 06/23/2017] [Accepted: 07/12/2017] [Indexed: 12/12/2022] Open
Abstract
Cholangitis is a serious life-threatening situation affecting the hepatobiliary system. This review provides an update regarding the clinical and pathological features of various forms of cholangitis. A comprehensive search was performed in the PubMed, Scopus, and Web of Knowledge databases. It was found that the etiology and pathogenesis of cholangitis are heterogeneous. Cholangitis can be categorized as primary sclerosing (PSC), secondary (acute) cholangitis, and a recently characterized form, known as IgG4-associated cholangitis (IAC). Roles of genetic and acquired factors have been noted in development of various forms of cholangitis. PSC commonly follows a chronic and progressive course that may terminate in hepatobiliary neoplasms. In particular, PSC commonly has been associated with inflammatory bowel disease. Bacterial infections are known as the most common cause for AC. On the other hand, IAC has been commonly encountered along with pancreatitis. Imaging evaluation of the hepatobiliary system has emerged as a crucial tool in the management of cholangitis. Endoscopic retrograde cholangiography, magnetic resonance cholangiopancreatography and endoscopic ultrasonography comprise three of the modalities that are frequently exploited as both diagnostic and therapeutic tools. Biliary drainage procedures using these methods is necessary for controlling the progression of cholangitis. Promising results have been reported for the role of antibiotic treatment in management of AC and PSC; however, immunosuppressive drugs have also rendered clinical responses in IAC. With respect to the high rate of complications, surgical interventions in patients with cholangitis are generally restricted to those patients in whom other therapeutic approaches have failed.
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Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis - a comprehensive review. J Hepatol 2017; 67:1298-1323. [PMID: 28802875 DOI: 10.1016/j.jhep.2017.07.022] [Citation(s) in RCA: 558] [Impact Index Per Article: 69.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/04/2017] [Revised: 07/15/2017] [Accepted: 07/22/2017] [Indexed: 02/07/2023]
Abstract
Primary sclerosing cholangitis (PSC) is a rare disorder characterised by multi-focal bile duct strictures and progressive liver disease. Inflammatory bowel disease is usually present and there is a high risk of cholangiocarcinoma and colorectal cancer. Most patients ultimately require liver transplantation, after which disease recurrence may occur. With limited therapeutic options and a lack of proven surveillance strategies, patients currently have significant unmet needs. In the present seminar, we provide a comprehensive review of the status of the field. We emphasise developments related to patient stratification and disease behaviour, and provide an overview of management options from a practical, patient-centered perspective. We survey advances made in the understanding of PSC pathogenesis and summarise the ongoing efforts to develop an effective therapy based on these insights.
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Affiliation(s)
- Tom H Karlsen
- Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Institute of Clinical Medicine, University of Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway.
| | - Trine Folseraas
- Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; Research Institute of Internal Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway
| | - Douglas Thorburn
- UCL Institute for Liver and Digestive Health, Division of Medicine, University College London, UK; Sheila Sherlock Liver Centre, Royal Free London NHS Foundation Trust, London, UK
| | - Mette Vesterhus
- Norwegian PSC Research Center, Department of Transplantation Medicine, Division of Surgery, Inflammatory Medicine and Transplantation, Oslo University Hospital Rikshospitalet, Oslo, Norway; National Centre for Ultrasound in Gastroenterology, Haukeland University Hospital, Bergen, Norway
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Alberca de Las Parras F, Egea Valenzuela J, Carballo Álvarez F. Bleeding risk in endoscopic retrograde cholangiopancreatography. Impact of the use of antithrombotic drugs. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2017; 109:202-210. [PMID: 28112960 DOI: 10.17235/reed.2017.4358/2016] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
AIMS To analyze the risk factors for hemorrhage during endoscopic retrograde cholangiopancreatography and the impact of antithrombotic drugs. MATERIAL AND METHODS Data sources: papers indexed in PubMed have been reviewed, as well as those found during the analysis of the bibliography of meta-analysis and reviews. SELECTION CRITERIA the references have been firstly evaluated by review of the abstract. After selecting the most significant articles (mainly randomized trials and well-designed case series) these have been deeply analyzed. Evaluation of the studies and synthesis: criteria by the Oxford Centre for Evidence-Based Medicine have been used for the analysis of the references and elaboration of evidence levels. RESULTS Seven hundred and sixty-five references were found, 753 in PubMed and the Cochrane Library. Twelve studies were selected during the analysis of other published articles (systematic reviews, meta-analysis and clinical practice guidelines). After analyzing the title or the abstract, 655 studies were excluded. Finally, 83 high quality trials or descriptive studies have been included in the analysis. CONCLUSION Seven conclusions regarding the risk factors for bleeding and the impact of antithrombotic drugs have been defined.
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Affiliation(s)
| | - Juan Egea Valenzuela
- Unidad de Gestión Clínica digestivo, Hospital Clínico Universitario Virgen de la Arrixaca
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Abstract
Primary sclerosing cholangitis (PSC) remains a rare but potentially devastating chronic, cholestatic liver disease. PSC causes obstruction of intra- and/or extra-hepatic bile ducts by inflammation and fibrosis, leading to biliary obstruction, cirrhosis and portal hypertension with all associated sequelae. The most dreaded consequence of PSC is cholangiocarcinoma, occurring in 10-20% of patients with PSC, and with population-based estimates of a 398-fold increased risk of cholangiocarcinoma in patients with PSC compared to the general population. We use the 4-D approach to endoscopic evaluation and management of PSC based on currently available evidence. After laboratory testing with liver chemistries and high-quality cross-sectional imaging with MRCP, the first D is Dominant stricture diagnosis and evaluation. Second, Dilation of strictures found during ERCP is performed using balloon dilation to as many segments as possible. Third, Dysplasia and cholangiocarcinoma diagnosis is performed by separated brushings for conventional cytology and fluorescence in situ hybridization (FISH), and consideration for direct cholangioscopy with SpyGlass™. Fourt and finally, Dosing of antibiotics is critical to prevent peri-procedural cholangitis. The aim of this review article is to explore endoscopic tools and techniques for the diagnosis and management of PSC and provide a practical approach for clinicians.
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Affiliation(s)
- Jodie A Barkin
- University of Miami, Leonard M. Miller School of Medicine, Department of Medicine, Division of Gastroenterology. Miami, Florida, USA
| | - Cynthia Levy
- University of Miami, Leonard M. Miller School of Medicine, Department of Medicine, Division of Hepatology. Miami, Florida, USA
| | - Enrico O Souto
- University of Miami, Leonard M. Miller School of Medicine, Department of Medicine, Division of Gastroenterology. Miami, Florida, USA
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Navaneethan U, Lourdusamy D, Gutierrez NG, Zhu X, Vargo JJ, Parsi MA. New approach to decrease post-ERCP adverse events in patients with primary sclerosing cholangitis. Endosc Int Open 2017; 5:E710-E717. [PMID: 28791317 PMCID: PMC5546902 DOI: 10.1055/s-0043-102398] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/27/2016] [Accepted: 12/30/2016] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND AND STUDY AIMS Endoscopic retrograde cholangiopancreatography (ERCP) is often performed in patients with primary sclerosing cholangitis (PSC). Our aim was to validate a treatment approach with the objective of decreasing ERCP related adverse events (AEs). PATIENTS AND METHODS All patients who had undergone ERCP for PSC during the period from 2002 - 2012 were identified (group I). This group had traditional ERCP (no bile aspiration prior to contrast injection with balloon dilation and stent placement for treatment of dominant strictures). To decrease ERCP-related AEs, we changed the ERCP approach in which bile aspiration was performed prior to contrast injection and balloon dilation alone was performed for treatment of dominant strictures. This was tested prospectively in all patients undergoing ERCP for PSC from 2012 - 2014 (group II). RESULTS The risk of overall AEs and cholangitis was relatively less in group II compared with group I [(2.1 % vs. 10.3 %; P = .38) and (0 % vs. 4.4 %; P = .68)]. On bivariate analysis, change in ERCP approach was associated with decreased risk of post-procedure cholangitis (0 % vs. 10.2 %, P = .03) and overall AE (0 % vs. 18.6 %, P = .03). There were no AEs in 22/46 patients in group II who had bile aspiration with balloon dilation. On multivariate analysis, only biliary stent placement was associated with increased risk of AEs (OR 4.10 (1.32 - 12.71); P = .02) and cholangitis (OR 5.43, 1.38 - 21.38; P = .02) respectively. CONCLUSION Biliary aspiration and avoidance of stenting approach after dilation of strictures during ERCP in PSC patients appears to be associated with decreased risk of cholangitis and overall AEs. Future prospective randomized controlled trials are needed to validate our observation.
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Affiliation(s)
- Udayakumar Navaneethan
- Center for Interventional Endoscopy, Orlando, Florida, United States,Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, United States,Corresponding author Udayakumar Navaneethan, MD FACP Center for Interventional EndoscopyAssistant Professor of Internal MedicineUniversity of Central Florida College of MedicineFlorida Hospital601 E Rollins StreetOrlando, FL 32803
| | - Dennisdhilak Lourdusamy
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, United States
| | - Norma G Gutierrez
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, United States
| | - Xiang Zhu
- Center for Interventional Endoscopy, Orlando, Florida, United States
| | - John J Vargo
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, United States
| | - Mansour A Parsi
- Department of Gastroenterology and Hepatology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, United States
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Björnsson ES. Primary sclerosing cholangitis: best practices for diagnosis. Expert Opin Orphan Drugs 2017. [DOI: 10.1080/21678707.2017.1358163] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 10/19/2022]
Affiliation(s)
- Einar S. Björnsson
- Department of Internal Medicine, Faculty of Medicine, University of Iceland, Reykjavik, Iceland
- Division of Gastroenterology and Hepatology, The National University Hospital of Iceland
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Asia-Pacific consensus guidelines for endoscopic management of benign biliary strictures. Gastrointest Endosc 2017; 86:44-58. [PMID: 28283322 DOI: 10.1016/j.gie.2017.02.031] [Citation(s) in RCA: 40] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/12/2016] [Accepted: 02/23/2017] [Indexed: 12/11/2022]
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Aabakken L, Karlsen TH, Albert J, Arvanitakis M, Chazouilleres O, Dumonceau JM, Färkkilä M, Fickert P, Hirschfield GM, Laghi A, Marzioni M, Fernandez M, Pereira SP, Pohl J, Poley JW, Ponsioen CY, Schramm C, Swahn F, Tringali A, Hassan C. Role of endoscopy in primary sclerosing cholangitis: European Society of Gastrointestinal Endoscopy (ESGE) and European Association for the Study of the Liver (EASL) Clinical Guideline. J Hepatol 2017; 66:1265-1281. [PMID: 28427764 DOI: 10.1016/j.jhep.2017.02.013] [Citation(s) in RCA: 67] [Impact Index Per Article: 8.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/14/2017] [Accepted: 02/14/2017] [Indexed: 02/06/2023]
Abstract
This guideline is an official statement of the European Society of Gastrointestinal Endoscopy (ESGE) and of the European Association for the Study of the Liver (EASL) on the role of endoscopy in primary sclerosing cholangitis. The Grading of Recommendations Assessment, Development and Evaluation (GRADE) system was adopted to define the strength of recommendations and the quality of evidence. Main recommendations.
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Razzak A, Kozarek R. Diagnosis and endoscopic management of primary sclerosing cholangitis. TECHNIQUES IN GASTROINTESTINAL ENDOSCOPY 2016; 18:158-167. [DOI: 10.1016/j.tgie.2016.05.003] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/18/2023]
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Endoscopic retrograde cholangiopancreatography-related adverse events in patients with type 1 autoimmune pancreatitis. Pancreatology 2015; 16:78-82. [PMID: 26626204 DOI: 10.1016/j.pan.2015.10.011] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/24/2015] [Revised: 09/23/2015] [Accepted: 10/31/2015] [Indexed: 12/11/2022]
Abstract
BACKGROUND Endoscopic retrograde cholangiopancreatography (ERCP) is frequently performed for the diagnosis and treatment of type 1 autoimmune pancreatitis (AIP). However, the prevalence of ERCP-related adverse events in patients with type 1 AIP has not been evaluated. We aimed to clarify the feasibility of ERCP in patients with type 1 AIP. METHODS We retrospectively reviewed 82 consecutive ERCP procedures performed in patients with type 1 AIP from 2004 to 2014 in one university hospital and three tertiary-care referral centers. One hundred four ERCP procedures in chronic pancreatitis and 1123 in non-AIP cohort were enrolled as control groups. We compared the incidence of post-ERCP pancreatitis (PEP) between type 1 AIP and control groups. We evaluated the incidence of ERCP-related adverse events and various predictive factors for hyperamylasemia after ERCP. RESULTS Pancreatography and cholangiography by ERCP were obtained in 78 (95.1%) and 76 (92.7%) patients, respectively. The incidence of PEP, cholangitis, and bleeding was 1.2% (1/82), 0%, and 1.2%, respectively. PEP occurred in type 1 AIP patient with diffuse parenchymal imaging, and the severity was mild. The incidences of PEP were 2.9% (3/104) and 5.4% (61/1123) in chronic pancreatitis and normal cohort, respectively. The incidence of PEP was slightly lower in type 1 AIP than non-AIP cohort (1.2% vs 5.8%, p = 0.119). There were no significant predictive factors for hyperamylasemia after ERCP in type 1 AIP. CONCLUSIONS The incidence of ERCP-related adverse events is low in patients with type 1 AIP. ERCP-related procedures are feasible in the diagnosis and treatment of AIP.
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Tharian B, George NE, Tham TCK. What is the current role of endoscopy in primary sclerosing cholangitis? World J Gastrointest Endosc 2015; 7:920-7. [PMID: 26265986 PMCID: PMC4530326 DOI: 10.4253/wjge.v7.i10.920] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/06/2015] [Revised: 03/21/2015] [Accepted: 07/11/2015] [Indexed: 02/05/2023] Open
Abstract
Endoscopy has important roles in the management of primary sclerosing cholangitis (PSC), ranging from narrowing down the differential diagnoses, screening for complications, determining prognosis and therapy. While the need for a diagnostic endoscopic retrograde cholangiopancreatography (ERCP) may be obviated by a positive magnetic resonance cholangiopancreatography (MRCP), a negative MRCP does not exclude PSC and may therefore necessitate an ERCP, which is traditionally regarded as the gold standard. In this editorial we have not covered the endoscopic management of inflammatory bowel disease in the context of PSC nor of endoscopic surveillance and treatment of portal hypertension complicating PSC.
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