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Naitoh I, Yoshida M, Nakazawa T. Endoscopic diagnosis of immunoglobulin G4-related sclerosing cholangitis. Dig Endosc 2025. [PMID: 40256978 DOI: 10.1111/den.15039] [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: 12/31/2024] [Accepted: 04/02/2025] [Indexed: 04/22/2025]
Abstract
Immunoglobulin G4 (IgG4)-related sclerosing cholangitis (IgG4-SC) is a distinct form of sclerosing cholangitis frequently associated with autoimmune pancreatitis and is recognized as a biliary manifestation of IgG4-related disease. Endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasonography (EUS) are key diagnostic modalities for IgG4-SC. Cholangiocarcinoma and primary sclerosing cholangitis (PSC) are significant mimickers of IgG4-SC. ERCP is employed to evaluate narrowing of the bile duct, with cholangiograms of IgG4-SC classified into four types. This cholangiographic classification is crucial for differential diagnosis. Characteristic cholangiographic findings of IgG4-SC include diffuse or segmental strictures of the intrahepatic or extrahepatic bile ducts and intrahepatic strictures associated with autoimmune pancreatitis. ERCP is particularly useful for differentiating IgG4-SC from PSC because their cholangiographic features differ. EUS and intraductal ultrasonography (IDUS) are used to assess thickening of the bile duct wall. Characteristic IDUS findings in IgG4-SC include circular and symmetrical wall thickening, smooth outer and inner margins, and homogeneous internal echoes at stricture sites. Additionally, bile duct wall thickening at nonstricture sites is a typical IDUS feature of IgG4-SC. Bile duct biopsy is used to evaluate pathological findings, although its diagnostic yield for IgG4-SC is limited; its primary role is to exclude malignant biliary strictures. Duodenal papilla biopsy serves as a supplementary diagnostic tool for IgG4-SC. EUS and tissue acquisition also aid in diagnosing autoimmune pancreatitis as part of other organ involvement. Thus, endoscopic techniques play critical roles in the diagnosis of IgG4-SC.
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Affiliation(s)
- Itaru Naitoh
- Department of Gastroenterology, Nagoya City University Midori Municipal Hospital, Aichi, Japan
| | - Michihiro Yoshida
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan
| | - Takahiro Nakazawa
- Department of Gastroenterology, Nagoya City University Graduate School of Medical Sciences, Aichi, Japan
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Vanhanen E, Ovissi A, Kolho KL, Tenca A. Assessment of disease severity with magnetic resonance cholangiography in pediatric-onset primary sclerosing cholangitis. J Pediatr Gastroenterol Nutr 2024; 79:644-651. [PMID: 39014988 DOI: 10.1002/jpn3.12319] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/08/2024] [Revised: 06/18/2024] [Accepted: 07/02/2024] [Indexed: 07/18/2024]
Abstract
OBJECTIVES Magnetic resonance cholangiopancreaticography (MRCP) has supplanted endoscopic retrograde cholangiopancreaticography (ERCP) as the preferred imaging modality for primary sclerosing cholangitis (PSC). However, data about the accuracy of MRCP in assessing disease severity are limited, particularly in children. We assessed the accuracy of MRCP in disease severity evaluation and investigated the correlation between imaging findings and biochemical parameters (including the multivariate risk index SCOPE) in patients with pediatric-onset PSC. METHODS We included 36 patients with PSC (median age: 16) who had MRCP and ERCP performed within 4-month intervals. Two experts, blinded to ERCP findings, evaluated the bile duct changes in consensus using the Modified Amsterdam PSC Score. The agreement between MRCP and ERCP evaluations was tested with weighted kappa statistics and the correlation between disease severity and biochemical parameters with Spearman's rank correlation. RESULTS The agreement between MRCP and ERCP was good for extrahepatic (weighted kappa 0.69; 95% confidence of interval [CI] 0.53-0.84) but fair for intrahepatic (weighted kappa 0.35; 95% CI 0.14-0.56) bile ducts. Intrahepatic and extrahepatic MRCP scores correlated with APRI (ρ = 0.42, p = 0.020 and ρ = 0.39, p = 0.033, respectively), while extrahepatic MRCP score also correlated with biliary neutrophils (ρ = 0.36, p = 0.035). We found a good correlation between the SCOPE index and intrahepatic MRCP score (ρ = 0.53, p = 0.004), and extrahepatic MRCP score (ρ = 0.57, p = 0.001). CONCLUSIONS MRCP is accurate at evaluating the severity of extrahepatic bile duct changes in pediatric-onset PSC but tends to underestimate intrahepatic changes. The SCOPE index's robust correlation with imaging scores supports its role as a comprehensive diagnostic tool, outperforming individual laboratory metrics.
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Affiliation(s)
- Enni Vanhanen
- Department of Radiology, HUS Diagnostic Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Ali Ovissi
- Department of Radiology, HUS Diagnostic Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Kaija-Leena Kolho
- Department of Paediatric Gastroenterology, Children's Hospital, University of Helsinki and University Hospital, Helsinki, Finland
| | - Andrea Tenca
- Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
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Okamoto K, Hijioka S, Nagashio Y, Okada M, Ohba A, Maruki Y, Kondo S, Morizane C, Ueno H, Okusaka T. Immune-related adverse event-associated sclerosing cholangitis due to immune checkpoint inhibitors: imaging findings and treatments. Jpn J Clin Oncol 2024; 54:887-894. [PMID: 38715325 DOI: 10.1093/jjco/hyae060] [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: 02/26/2024] [Accepted: 05/02/2024] [Indexed: 08/15/2024] Open
Abstract
OBJECTIVES Immune-related adverse event-sclerosing cholangitis caused by treatment with immune checkpoint inhibitors is rare, and the diagnostic criteria and treatment strategy remain unclear. In this study, we confirmed the clinicopathological features of immune-related adverse event-sclerosing cholangitis and clarified its diagnosis and appropriate management. METHODS We retrospectively evaluated 10 patients diagnosed with immune-related adverse event-sclerosing cholangitis and identified by electronic database searches. RESULTS Blood tests revealed liver dysfunction with a predominance of biliary tract enzymes in all patients; however, jaundice was present in only one patient. Contrast-enhanced computed tomography revealed diffuse hypertrophy of the extrahepatic bile duct wall as the most frequent finding; however, endoscopic retrograde cholangiopancreatography showed various imaging features, such as the pruned-tree appearance of intrahepatic bile ducts, in all patients. Transpapillary bile duct biopsy showed inflammatory cell infiltration using immunostaining, with a predominance of cluster of differentiation 8-positive T cells in 63% of the cases. Initial steroid therapy was effective in two cases. Mycophenolate mofetil and tacrolimus were used in steroid-refractory cases. Although six patients showed improvements, all of the remaining patients died owing to immune-related adverse event-sclerosing cholangitis. CONCLUSIONS Various bile duct imaging findings of immune-related adverse event-sclerosing cholangitis were revealed; transpapillary bile duct biopsy may be useful in the diagnosis of immune-related adverse event-sclerosing cholangitis. Despite the combination of multiple immunosuppressive agents, prognosis of immune-related adverse event-sclerosing cholangitis remains poor. Longer follow-up and larger clinical studies are necessary to establish its treatment strategy.
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Affiliation(s)
- Kohei Okamoto
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Susumu Hijioka
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Yoshikuni Nagashio
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Mao Okada
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Akihiro Ohba
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Yuta Maruki
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Shunsuke Kondo
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Chigusa Morizane
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Hideki Ueno
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
| | - Takuji Okusaka
- Department of Hepatobiliary and Pancreatic Oncology, National Cancer Center Hospital, Tokyo, Japan
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4
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Vanhanen E, Ovissi A, Kolho KL, Tenca A. Magnetic resonance cholangiography in the diagnosis of dominant strictures in pediatric-onset primary sclerosing cholangitis. Dig Liver Dis 2023; 55:1496-1501. [PMID: 37286450 DOI: 10.1016/j.dld.2023.05.024] [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: 02/13/2023] [Revised: 04/25/2023] [Accepted: 05/20/2023] [Indexed: 06/09/2023]
Abstract
BACKGROUND Magnetic resonance cholangiopancreaticography (MRCP) has become the primary imaging modality in primary sclerosing cholangitis (PSC). Endoscopic retrograde cholangiopancreaticography (ERCP) is recommended when a dominant stricture (DS) of bile ducts is suspected in MRCP. However, MRCP criteria for DS are lacking. AIMS To evaluate the diagnostic accuracy of MRCP in the diagnosis of DS in patients with pediatric-onset PSC. METHODS ERCP and MRCP images of patients with pediatric-onset PSC (n=36) were evaluated for the presence of DS applying the diameter-based ERCP criteria. The diagnostic accuracy of MRCP in detecting DS was calculated using ERCP as the gold standard. RESULTS The sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, and accuracy of MRCP for detecting DS were 62%, 89%, 5.6, 0.43, and 81%. Most common reasons for incongruent ERCP/MRCP assessment were (1) MRCP stenosis not fulfilling the diameter criteria of ERCP, resulting in false negative MRCP evaluation, and (2) lack of filling pressure in MRCP, resulting in false positive MRCP evaluation. CONCLUSION The high positive likelihood ratio of MRCP in detecting DS suggests that MRCP is a useful tool in the follow-up of PSC. However, diameter limits of DS should probably be less strict in MRCP than in ERCP.
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Affiliation(s)
- Enni Vanhanen
- Department of Radiology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Ali Ovissi
- Department of Radiology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
| | - Kaija-Leena Kolho
- Department of Paediatric Gastroenterology, Children's Hospital, University of Helsinki and University Hospital, Helsinki, Finland
| | - Andrea Tenca
- Abdominal Center, Endoscopy Unit, University of Helsinki and Helsinki University Hospital, POB 340, 00029 HUS, Helsinki, Finland.
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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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Abstract
PURPOSE OF REVIEW Magnetic resonance cholangiopancreatography (MRCP) has become the reference examination for the exploration of the biliary tract and has replaced endoscopic cholangiography for the analysis of the biliary tract because of its equivalent performance and its noninvasive character. RECENT FINDINGS Based on the International Primary Sclerosing Cholangitis (PSC) Study Group recommendations for MR imaging in PSC, two protocols can be distinguished for the imaging of biliary tract: a basic protocol and a more complete protocol. It is essential to know the main pitfalls in order not to wrongly describe biliary anomalies. In addition to the excellent performance of MR imaging with MRCP in analyzing the anatomy and the anomalies of the biliary tree, complementary techniques have recently been developed. Several MR prognostic factors have been described. New hepato-specific contrast agents are now available for assessment of the general and segmental liver function. MR Elastography and Diffusion-weighted MR sequences are accurate to evaluate the degree of hepatic fibrosis. Finally, images obtained in MRCP can be postprocessed by a software that will analyze and model the biliary tree in order to quantitatively evaluate the biliary system. SUMMARY Magnetic resonance imaging with its recent developments becomes by now an essential tool for the evaluation of biliary diseases.
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Di Giorgio A, Vergani D, Mieli-Vergani G. Cutting edge issues in juvenile sclerosing cholangitis. Dig Liver Dis 2022; 54:417-427. [PMID: 34289942 DOI: 10.1016/j.dld.2021.06.028] [Citation(s) in RCA: 6] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2021] [Revised: 06/08/2021] [Accepted: 06/23/2021] [Indexed: 12/11/2022]
Abstract
Sclerosing cholangitis (SC) is a rare chronic disorder characterised by inflammation and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts. Diagnosis is based on cholangiogram showing bile duct dilatation, narrowing and obliteration of the biliary tree, and histologically, on the presence of inflammatory bile duct damage leading to periductal fibrosis. In children the most common SC is associated with strong autoimmune features, overlapping with those of autoimmune hepatitis (AIH); this form is known as autoimmune sclerosing cholangitis, ASC. Conversely, primary SC (PSC), a condition in which the term "primary" indicates that aetiology and pathogenesis are unknown, is rare in paediatrics. Secondary SC (SSC) defines a cholangiopathy associated with an identifiable aetiology such as immunodeficiencies, infections or haematological disorders. ASC and PSC are strongly associated with inflammatory bowel disease (IBD). ASC responds biochemically well to immunosuppressive drugs and ursodeoxycholic acid (UDCA). Primary forms are exclusively managed with oral UDCA, while in the secondary forms the medical treatment depends on the underlying aetiology. Despite treatment, SC often progresses to biliary cirrhosis and end-stage liver disease requiring liver transplantation. The disease can recur after transplant. Better understanding of pathogenic mechanisms and better treatment modalities are needed to improve the prognosis of this invalidating hepatic disorder.
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Affiliation(s)
- Angelo Di Giorgio
- Paediatric Hepatology, Gastroenterology and Transplantation, Hospital Papa Giovanni XXIII, Bergamo, Italy.
| | - Diego Vergani
- King's College London Faculty of Life Sciences and Medicine, Institute of Liver Studies, Mowat Labs King's College Hospital, London, United Kingdom
| | - Giorgina Mieli-Vergani
- King's College London Faculty of Life Sciences and Medicine, Institute of Liver Studies, Mowat Labs King's College Hospital, London, United Kingdom; Paediatric Liver, Gastrointestinal, and Nutrition Centre, King's College Hospital, London, United Kingdom
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8
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Stremmel W, Lukasova M, Weiskirchen R. The neglected biliary mucus and its phosphatidylcholine content: a putative player in pathogenesis of primary cholangitis-a narrative review article. ANNALS OF TRANSLATIONAL MEDICINE 2021; 9:738. [PMID: 33987436 PMCID: PMC8106090 DOI: 10.21037/atm-20-3591] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 12/12/2022]
Abstract
Primary sclerosing cholangitis (PSC) is a rare progressive cholangitis resulting in cirrhosis and cholangiocellular carcinoma. The pathogenesis is unclear and an effective medical therapy is not available. It is highly associated to ulcerative colitis for which recently a disturbance of the tight junction (TJ) barrier has been claimed as etiologic feature. Genetic mouse models with intestinal TJ disruption showed a defective transport of phosphatidylcholine (PC) to intestinal mucus. Consequently, an ulcerative colitis phenotype developed. In the present study we evaluate whether there is also a paracellular transport of PC through TJ to the apical side of cholangiocytes. As in ulcerative colitis, a TJ defect could lead to deficient PC in biliary mucus. It would impair the protective barrier against aggressive bile acids in bile. Indeed with polarized biliary tumor cells a vectorial transport of PC from basal to luminal side was demonstrated using a transwell culture system. PC was not taken up by the cells but moved paracellularly via TJ to the apical side driven by luminal HCO3- generated by the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and the anion exchange protein 2 (AE2). If such a TJ-mediated PC translocation to the apical surface of cholangiocytes could be disrupted in a genetic mouse model, a PSC phenotype would be expected. With such an experimental model functional operative therapies can be evaluated. We propose that disruption of TJ mediated paracellular transport of PC to the apical side of cholangiocytes could lead to biliary mucus PC depletion. This may be a pathogenetic factor for development of PSC.
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Affiliation(s)
| | - Martina Lukasova
- Pharmacy of University Clinics of Heidelberg, Heidelberg, Germany
| | - Ralf Weiskirchen
- Institute of Molecular Pathobiochemistry, Experimental Gene Therapy and Clinical Chemistry, RWTH University Hospital Aachen, Aachen, Germany
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Kumar N, Poddar U, Yadav R, Lal H, Pani K, Yachha SK, Srivastava A, Pandey R. Autoimmune Sclerosing Cholangitis in Children: A Prospective Case-Control Study. Pediatr Gastroenterol Hepatol Nutr 2021; 24:154-163. [PMID: 33833971 PMCID: PMC8007839 DOI: 10.5223/pghn.2021.24.2.154] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/24/2020] [Revised: 09/07/2020] [Accepted: 11/06/2020] [Indexed: 12/11/2022] Open
Abstract
PURPOSE In children overlap of autoimmune hepatitis (AIH) and primary sclerosing cholangitis is labelled as autoimmune sclerosing cholangitis (ASC). The only prospective pediatric study showed a high prevalence of ASC by using endoscopic retrograde cholangiopancreatography. Aims of our study were to find the prevalence of ASC by using magnetic resonance cholangiography (MRC) in AIH and in non-AIH cirrhosis and to compare clinical presentation and outcome of AIH and ASC. METHODS Prospectively we did MRC in 38 children with AIH (cases) and 19 disease controls (Wilson disease). Multiple biliary strictures with proximal dilatation on MRC were taken as definitive changes of ASC. Detail clinical, laboratory parameters, liver histopathology and treatment outcome were recorded. RESULTS The median age of cases was 11.5 (3-18) years, 22 (57.9%) were girls and 28 (73.7%) were diagnosed as type 1 AIH. MRC was done in 11 children (28.9%) at the time of diagnosis and in 27 (71.1%) after a median follow-up of 2.5 (0.3-10) years. Abnormal MRC changes were seen in 14/38 (36.8%) of AIH and 8/19 (42.1%) of controls. However, definite changes of ASC were present in four (10.5%) children in AIH and none in controls. None of the clinical, laboratory, histological parameters and treatment response were significantly different between ASC and AIH groups. CONCLUSION The prevalence of ASC in children with AIH was just 10.5%. We suggest MRC in select group with cholestatic features, inflammatory bowel disease and in those who showed poor response to immunosuppression instead of all children with AIH.
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Affiliation(s)
- Nagendra Kumar
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Ujjal Poddar
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Rajnikant Yadav
- Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Hira Lal
- Department of Radiodiagnosis, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Krushna Pani
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Surender Kumar Yachha
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Anshu Srivastava
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
| | - Rakesh Pandey
- Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India
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Culver EL, Bungay HK, Betts M, Forde C, Buchel O, Manganis C, Warren BF, Cummings FR, Keshav S, Travis SPL, Chapman RW. Prevalence and long-term outcome of sub-clinical primary sclerosing cholangitis in patients with ulcerative colitis. Liver Int 2020; 40:2744-2757. [PMID: 32841490 DOI: 10.1111/liv.14645] [Citation(s) in RCA: 17] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/17/2020] [Revised: 07/22/2020] [Accepted: 08/12/2020] [Indexed: 02/13/2023]
Abstract
BACKGROUND Primary sclerosing cholangitis (PSC) is closely associated with inflammatory bowel disease, particularly ulcerative colitis (UC), with an increased risk of biliary and colorectal malignancy. We sought to clarify the prevalence, characteristics and long-term outcome of sub-clinical PSC diagnosed by magnetic resonance cholangiogram (MRC) in patients with UC and normal liver biochemistry, with or without colorectal dysplasia (CRD). METHODS In this prospective case-control study, 70 patients with UC and normal liver function (51 extensive UC, 19 CRD), 28 healthy volunteers (negative controls) and 28 patients with PSC and cholestasis (positive controls) underwent MRC and blood evaluation. MRC scans were interpreted blindly by two radiologists who graded individually, the scans as definitive for PSC, possible for PSC or normal. Clinical outcome was assessed by blood monitoring, abdominal imaging and endoscopic surveillance. RESULTS 7/51 (14%) with extensive UC and 4/19 (21%) with CRD had biliary abnormalities on MRC consistent with PSC. 7/11 (64%) with sub-clinical PSC had isolated intrahepatic duct involvement. Sub-clinical PSC was associated with advanced age (P = .04), non-smoking (P = .03), pANCA (P = .04), quiescent colitis (P = .02), absence of azathioprine (P = .04) and high-grade CRD (P = .03). Inter-observer (kappa = 0.88) and intra-observer (kappa = 0.96) agreement for MRC interpretation was high. No negative controls were assessed as definite PSC, 4/28 were considered on blinding as possible PSC. During follow-up of sub-clinical PSC (median 10.1(3.1-11.9) years), four patients developed abnormal liver biochemistry, two had radiological progression of PSC and seven developed malignancy, including two biliary and one colorectal carcinoma. CONCLUSIONS Prevalence of sub-clinical PSC appears high in patients with extensive UC and normal liver biochemistry, with or without CRD. Disease progression and malignancy were identified on long-term follow-up. MRC should be considered for all patients with extensive UC or CRD to stratify surveillance.
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Affiliation(s)
- Emma L Culver
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
| | - Helen K Bungay
- Radiology Department, John Radcliffe Hospital and Churchill Hospital, Oxford, UK
| | - Margaret Betts
- Radiology Department, John Radcliffe Hospital and Churchill Hospital, Oxford, UK
| | - Colm Forde
- Radiology Department, John Radcliffe Hospital and Churchill Hospital, Oxford, UK.,Radiology Department, Queen Elizabeth Hospital, Birmingham, UK
| | - Otto Buchel
- Rondebosch Medical Centre, Cape Town, South Africa
| | - Charis Manganis
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
| | - Bryan F Warren
- Histopathology Department, John Radcliffe Hospital, Oxford, UK
| | - Fraser R Cummings
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK.,Gastroenterology Department, Southampton General Hospital, Southampton, UK
| | - Satish Keshav
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
| | - Simon P L Travis
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
| | - Roger W Chapman
- Translational Gastroenterology Unit, Nuffield Department of Medicine, John Radcliffe Hospital, University of Oxford, Oxford, UK
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11
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Mago S, Wu GY. Primary Sclerosing Cholangitis and Primary Biliary Cirrhosis Overlap Syndrome: A Review. J Clin Transl Hepatol 2020; 8:336-346. [PMID: 33083257 PMCID: PMC7562796 DOI: 10.14218/jcth.2020.00036] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/24/2020] [Revised: 07/21/2020] [Accepted: 07/24/2020] [Indexed: 12/12/2022] Open
Abstract
Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are slow progressive diseases which have been increasing in prevalence. The pathogeneses of PBC and PSC are incompletely understood but the underlying mechanisms appear to be fundamentally autoimmune in origin. Although PBC and PSC appear to be separate entities, overlap has been described. Diagnosis depends on a combination of serological markers, imaging, and pathological criteria. The mainstay of treatment has been ursodeoxycholic acid and in some cases of extrahepatic biliary obstruction and overlap disorder, endoscopic retrograde cholangiopancreatography has been useful.
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Affiliation(s)
- Sheena Mago
- Department of Medicine, Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
- Correspondence to: Sheena Mago, Department of Medicine, University of Connecticut Health Center, 263 Farmington Avenue, Farmington, CT 06030, USA. E-mail:
| | - George Y. Wu
- Department of Medicine, Division of Gastroenterology-Hepatology, University of Connecticut Health Center, Farmington, CT, USA
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12
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Editorial for "Quantitative MRCP Imaging: Accuracy, Repeatability, Reproducibility, and Cohort‐Derived Normative Ranges. J Magn Reson Imaging 2020; 52:821-822. [DOI: 10.1002/jmri.27110] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2020] [Accepted: 02/06/2020] [Indexed: 11/07/2022] Open
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13
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Manganis CD, Chapman RW, Culver EL. Review of primary sclerosing cholangitis with increased IgG4 levels. World J Gastroenterol 2020; 26:3126-3144. [PMID: 32684731 PMCID: PMC7336326 DOI: 10.3748/wjg.v26.i23.3126] [Citation(s) in RCA: 28] [Impact Index Per Article: 5.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/23/2019] [Revised: 04/07/2020] [Accepted: 06/05/2020] [Indexed: 02/06/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) is a chronic progressive liver disease. Sub-types of PSC have been described, most recently PSC with elevated serum and/or tissue IgG4 subclass. We aim to summarise the clinical phenotype, disease associations, differential diagnosis, response to therapy and pathogenic mechanisms underlying PSC-high IgG4 subtype. We reviewed PubMed, MEDLINE and Embase with the search terms "primary sclerosing cholangitis", "IgG4", and "IgG4-related sclerosing cholangitis (IgG4-SC)". Elevated serum IgG4 are found in up-to one-quarter, and abundant IgG4-plasma cell infiltrates in the liver and bile ducts are found in up-to one-fifth of PSC patients. This group have a distinct clinical phenotype, with some studies reporting a more aggressive course of liver and associated inflammatory bowel disease, compared to PSC-normal IgG4 and the disease mimic IgG4-SC. Distinguishing PSC-high IgG4 from IgG4-SC remains challenging, requiring careful assessment of clinical features, organ involvement and tissue morphology. Calculation of serum IgG4:IgG1 ratios and use of a novel IgG4:IgG RNA ratio have been reported to have excellent specificity to distinguish IgG4-SC and PSC-high IgG4 but require validation in larger cohorts. A role for corticosteroid therapy in PSC-high IgG4 remains unanswered, with concerns of increased toxicity and lack of outcome data. The immunological drivers underlying prominent IgG4 antibodies in PSC are incompletely defined. An association with PSC-high IgG4 and HLA class-II haplotypes (B*07, DRB1*15), T-helper2 and T-regulatory cytokines (IL4, IL10, IL13) and chemokines (CCL1, CCR8) have been described. PSC-high IgG4 have a distinct clinical phenotype and need careful discrimination from IgG4-SC, although response to immunosuppressive treatments and long-term outcome remains unresolved. The presence of IgG4 likely represents chronic activation to persistent antigenic exposure in genetically predisposed individuals.
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Affiliation(s)
- Charis D Manganis
- Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
| | - Roger W Chapman
- Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
| | - Emma L Culver
- Translational Gastroenterology Unit and Oxford NIHR Biomedical Research Centre, John Radcliffe Hospital, Oxford OX3 9DU, United Kingdom
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14
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Dumonceau JM, Delhaye M, Charette N, Farina A. Challenging biliary strictures: pathophysiological features, differential diagnosis, diagnostic algorithms, and new clinically relevant biomarkers - part 1. Therap Adv Gastroenterol 2020; 13:1756284820927292. [PMID: 32595761 PMCID: PMC7298429 DOI: 10.1177/1756284820927292] [Citation(s) in RCA: 14] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2020] [Accepted: 04/16/2020] [Indexed: 02/04/2023] Open
Abstract
It is frequently challenging to make the correct diagnosis in patients with biliary strictures. This is particularly important as errors may have disastrous consequences. Benign-appearing strictures treated with stents may later be revealed to be malignant and unnecessary surgery for benign strictures carries a high morbidity rate. In the first part of the review, the essential information that clinicians need to know about diseases responsible for biliary strictures is presented, with a focus on the most recent data. Then, the characteristics and pitfalls of the methods used to make the diagnosis are summarized. These include serum biomarkers, imaging studies, and endoscopic modalities. As tissue diagnosis is the only 100% specific tool, it is described in detail, including techniques for tissue acquisition and their yields, how to prepare samples, and what to expect from the pathologist. Tricks to increase diagnostic yields are described. Clues are then presented for the differential diagnosis between primary and secondary sclerosing cholangitis, IgG4-related sclerosing cholangitis, cholangiocarcinoma, pancreatic cancer, autoimmune pancreatitis, and less frequent diseases. Finally, algorithms that will help to achieve the correct diagnosis are proposed.
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Affiliation(s)
- Jean-Marc Dumonceau
- Department of Gastroenterology, Charleroi
University Hospitals, Chaussée de Bruxelles 140, Charleroi, 6042,
Belgium
| | - Myriam Delhaye
- Department of Gastroenterology,
Hepatopancreatology and GI Oncology, Erasme University Hospital, Brussels,
Belgium
| | - Nicolas Charette
- Department of Gastroenterology, Charleroi
University Hospitals, Charleroi, Belgium
| | - Annarita Farina
- Department of Medicine, Geneva University,
Geneva, Switzerland
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15
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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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16
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Satiya J, Mousa OY, Gupta K, Trivedi S, Oman SP, Wijarnpreecha K, Harnois DM, Corral JE. Diagnostic yield of magnetic resonance imaging for cholangiocarcinoma in primary sclerosing cholangitis: a meta-analysis. Clin Exp Hepatol 2020; 6:35-41. [PMID: 32166122 PMCID: PMC7062114 DOI: 10.5114/ceh.2020.93054] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/18/2019] [Accepted: 11/07/2019] [Indexed: 02/07/2023] Open
Abstract
AIM OF THE STUDY Combined magnetic resonance imaging and magnetic resonance cholangiopancreatography (MRI/MRCP) can identify biliary strictures and diagnose primary sclerosing cholangitis (PSC). Diagnosis of cholangiocarcinoma in patients with PSC remains challenging, and the accuracy of MRI/MRCP has not been completely established. We aimed to determine the sensitivity and specificity of MRI/MRCP in the diagnosis of cholangiocarcinoma among patients with PSC from the published literature. MATERIAL AND METHODS We searched Embase, PubMed, Cochrane, Scopus, ClinicalTrials.gov, and abstracts from relevant scientific meetings and performed a systematic review and meta-analysis to estimate the diagnostic yield of MRI/MRCP in patients with PSC. Sensitivity and specificity were calculated from pooled estimates of cholangiocarcinoma cases identified and lesions missed. Modifying variables were included in a meta-regression model. RESULTS Our literature search yielded 302 articles and 9 conference abstracts; 8 studies involving 846 liver patients from 5 countries were included in the final analysis. Of those, 531 had PSC and received MRI/MRCP. Thirty-six (6.8%) patients were diagnosed with cholangiocarcinoma (33 true positive, 3 false negative and 1 false positive). Pooled sensitivity was 98.9% (95% CI: 98.6-99.3%). Cholangiocarcinoma cases missed by MRI/MRCP were diagnosed as beading irregularities of the central hepatic ducts, or PSC-related diffuse stricture. Metaregression revealed that neither publication year, study design, nor sample size had a significant effect on observed cancer rates (p = 0.9, 0.3, and 0.3, respectively). CONCLUSIONS MRI/MRCP followed by endoscopic retrograde cholangiopancreatography (ERCP) is a sensitive and specific tool to diagnose cholangiocarcinoma among patients with PSC. Further research should estimate MRI/MRCP diagnostic accuracy for cholangiocarcinoma using prospective methodology and longer term outcomes.
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Affiliation(s)
- Jinendra Satiya
- Department of Internal Medicine, University of Miami/JFK Medical Center, West Palm Beach, FL, Unites States
| | - Omar Y Mousa
- Department of Hepatology, Mayo Clinic, Rochester, MN, Unites States
| | - Kapil Gupta
- Department of Internal Medicine, University of Miami/JFK Medical Center, West Palm Beach, FL, Unites States
| | - Shivani Trivedi
- Department of Internal Medicine, University of Miami/JFK Medical Center, West Palm Beach, FL, Unites States
| | - Sven P Oman
- Department of Gastroenterology, Mayo Clinic, Jacksonville, FL, Unites States
| | - Karn Wijarnpreecha
- Department of Gastroenterology, Mayo Clinic, Jacksonville, FL, Unites States
| | - Denise M Harnois
- Department of Gastroenterology, Mayo Clinic, Jacksonville, FL, Unites States
| | - Juan Enrique Corral
- Department of Gastroenterology, Mayo Clinic, Jacksonville, FL, Unites States
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17
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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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18
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Schiavon LDL, Ejima FH, Menezes MRD, Bittencourt PL, Moreira AM, Farias AQ, Chagas AL, Assis AMD, Mattos ÂZD, Salomão BC, Terra C, Martins FPB, Carnevale FC, Rezende GFDM, Paulo GAD, Pereira GHS, Leal Filho JMDM, Meneses JD, Costa LSND, Carneiro MDV, Álvares-DA-Silva MR, Soares MVA, Pereira OI, Ximenes RO, Durante RFS, Ferreira VA, Lima VMD. RECOMMENDATIONS FOR INVASIVE PROCEDURES IN PATIENTS WITH DISEASES OF THE LIVER AND BILIARY TRACT: REPORT OF A JOINT MEETING OF THE BRAZILIAN SOCIETY OF HEPATOLOGY (SBH), BRAZILIAN SOCIETY OF DIGESTIVE ENDOSCOPY (SOBED) AND BRAZILIAN SOCIETY OF INTERVENTIONAL RADIOLOGY AND ENDOVASCULAR SURGERY (SOBRICE). ARQUIVOS DE GASTROENTEROLOGIA 2019; 56:213-231. [PMID: 31460590 DOI: 10.1590/s0004-2803.201900000-42] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/08/2019] [Accepted: 04/12/2019] [Indexed: 12/12/2022]
Abstract
Liver and biliary tract diseases are common causes of morbidity and mortality worldwide. Invasive procedures are usually performed in those patients with hepatobiliary diseases for both diagnostic and therapeutic purposes. Defining proper indications and restraints of commonly used techniques is crucial for proper patient selection, maximizing positive results and limiting complications. In 2018, the Brazilian Society of Hepato-logy (SBH) in cooperation with the Brazilian Society of Interventional Radiology and Endovascular surgery (SOBRICE) and the Brazilian Society of Digestive Endoscopy (SOBED) sponsored a joint single-topic meeting on invasive procedures in patients with hepatobiliary diseases. This paper summarizes the proceedings of the aforementioned meeting. It is intended to guide clinicians, gastroenterologists, hepatologists, radiologists, and endoscopists for the proper use of invasive procedures for management of patients with hepatobiliary diseases.
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Affiliation(s)
- Leonardo de Lucca Schiavon
- Universidade Federal de Santa Catarina, Faculdade de Medicina, Departamento de Clínica Médica, Florianópolis, SC, Brasil
| | | | - Marcos Roberto de Menezes
- Instituto do Câncer do Estado de São Paulo, Setor de Diagnóstico por Imagem, São Paulo, SP, Brasil
- Instituto do Câncer do Estado de São Paulo da Faculdade de Medicina da Universidade de São Paulo, Serviço de Radiologia Intervencionista, São Paulo, SP, Brasil
| | | | - Aírton Mota Moreira
- Universidade de São Paulo, Faculdade de Medicina, Serviço de Radiologia Intervencionista do Instituto de Radiologia, São Paulo, SP, Brasil
| | - Alberto Queiroz Farias
- Universidade de São Paulo, Faculdade de Medicina, Departamento de Gastroenterologia, São Paulo, SP, Brasil
| | - Aline Lopes Chagas
- Universidade de São Paulo, Faculdade de Medicina, Departamento de Gastroenterologia, São Paulo, SP, Brasil
| | - André Moreira de Assis
- Universidade de São Paulo, Faculdade de Medicina, Serviço de Radiologia Intervencionista do Instituto de Radiologia, São Paulo, SP, Brasil
- Hospital Sírio-Libanês, São Paulo, SP, Brasil
| | - Ângelo Zambam de Mattos
- Universidade Federal de Ciências da Saúde de Porto Alegre, Programa de Pós-Graduação em Medicina: Hepatologia, RS, Brasil
| | | | - Carlos Terra
- Universidade do Estado do Rio de Janeiro, Faculdade de Medicina, Departamento de Gastroenterologia, RJ, Brasil
- Hospital Federal de Lagoa, Departamento de Gastroenterologia, Rio de Janeiro, RJ, Brasil
| | | | - Francisco Cesar Carnevale
- Instituto de Radiologia da Faculdade de Medicina da Universidade de São Paulo, Serviço de Radiologia Intervencionista, São Paulo, SP, Brasil
| | | | | | | | - Joaquim Maurício da Motta Leal Filho
- Instituto do Câncer do Estado de São Paulo da Faculdade de Medicina da Universidade de São Paulo, Serviço de Radiologia Intervencionista, São Paulo, SP, Brasil
| | - Juliana de Meneses
- Instituto Hospital de Base do Distrito Federal, Brasília, DF, Brasil
- Instituto Nacional do Câncer, Brasília, DF, Brasil
| | - Lucas Santana Nova da Costa
- Instituto Hospital de Base do Distrito Federal, Brasília, DF, Brasil
- Hospital Sírio-Libanês Unidade Brasília, Brasília, DF, Brasil
| | - Marcos de Vasconcelos Carneiro
- Hospital das Forças Armadas, Brasília, DF, Brasil
- Universidade Católica de Brasília, Curso de Medicina, Brasília, DF, Brasil
| | - Mário Reis Álvares-DA-Silva
- Universidade Federal do Rio Grande do Sul, Faculdade de Medicina, Departamento de Medicina Interna, Rio Grande do Sul, RS, Brasil
| | - Mayra Veloso Ayrimoraes Soares
- Hospital Sírio-Libanês Unidade Brasília, Brasília, DF, Brasil
- Universidade de Brasília, Serviço de Radiologia, Brasília, DF, Brasil
| | - Osvaldo Ignácio Pereira
- Instituto de Radiologia da Faculdade de Medicina da Universidade de São Paulo, Serviço de Radiologia Intervencionista, São Paulo, SP, Brasil
| | - Rafael Oliveira Ximenes
- Hospital das Clínicas da Universidade Federal de Goiás, Serviço de Gastroenterologia e Hepatologia, Goiás, GO, Brasil
| | | | - Valério Alves Ferreira
- Instituto Hospital de Base do Distrito Federal, Brasília, DF, Brasil
- Hospital Santa Marta, Brasília, DF, Brasil
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19
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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: 178] [Impact Index Per Article: 29.7] [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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20
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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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21
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Seo KI, Kang SB. [Hepatobiliary Manifestation of Inflammatory Bowel Disease]. THE KOREAN JOURNAL OF GASTROENTEROLOGY 2019; 73:248-259. [PMID: 31132831 DOI: 10.4166/kjg.2019.73.5.248] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/03/2019] [Revised: 05/05/2019] [Accepted: 05/12/2019] [Indexed: 12/13/2022]
Abstract
The hepatobiliary system is one of the most common sites of extraintestinal manifestation in patients with inflammatory bowel disease (IBD). The progression of IBD can lead to a primary hepatobiliary manifestation and can occur secondary to multiple drugs or accompanying viral infections. Primary sclerosing cholangitis is the representative hepatobiliary manifestation of IBD, particularly in ulcerative colitis. Although most agents used in the treatment of IBD are potentially hepatotoxic, the risk of serious hepatitis or liver failure is low. The prevalence of HBV and HCV in IBD is similar to the general population, but the clinical concern is HBV reactivation associated with immunosuppressive therapy. Patients undergoing cytotoxic chemotherapy or immunosuppressive therapy with a moderate to high risk of HBV reactivation require prophylactic antiviral therapy. On the other hand, HCV has little risk of reactivation. Patients with IBD are more likely to have nonalcoholic fatty liver disease than the general population and tend to occur at younger ages. IBD and cholelithiasis are closely related, especially in Crohn's disease.
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Affiliation(s)
- Kwang Il Seo
- Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea
| | - Sang-Bum Kang
- Division of Gastroenterology, Department of Internal Medicine, Daejeon St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Daejeon, Korea
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22
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[Immune-mediated cholangiopathies : Diagnostic and therapeutic challenges]. Radiologe 2019; 59:348-356. [PMID: 30874827 DOI: 10.1007/s00117-019-0513-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/27/2022]
Abstract
BACKGROUND Immune-mediated cholangiopathies comprise primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and IgG4-associated cholangitis (IAC). A common feature is the progressive destruction of bile ducts leading to cholestasis with fibrosis and cirrhosis of the liver over time. The diseases are mostly identified during routine laboratory testing. Clinical signs and symptoms such as pruritus, fatigue or jaundice are infrequent in the early stage. DIAGNOSIS The diagnostic work-up involves the patient's history, physical examination, serological tests, abdominal ultrasonography, magnetic resonance cholangiopancreatography (MRCP) and, where necessary, liver biopsy and genetic testing. THERAPY Ursodeoxycholic acid (UDCA) is an effective treatment of PBC. Second-line therapies in addition to UDCA for incomplete UDCA responders are obeticholic acid (OCA) and bezafibrate, whereby only OCA has received approval for this indication from American (Federal Drug Administration) and European (European Medicines Agency) authorities. In PSC, UDCA improves prognostic markers; dominant bile duct strictures are treated with endoscopic balloon dilatation. Despite therapy, liver transplantation is frequently necessary for PSC. The risk of developing cholangiocarcinoma, colon cancer, and gallbladder cancer is increased for patients with PSC. In contrast to PBC and PSC, IAC responds well to corticosteroids. Disease relapse, however, is common, making long-term treatment with low-dose prednisolone or azathioprine necessary.
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23
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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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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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25
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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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26
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Schramm C, Eaton J, Ringe KI, Venkatesh S, Yamamura J. Recommendations on the use of magnetic resonance imaging in PSC-A position statement from the International PSC Study Group. Hepatology 2017; 66:1675-1688. [PMID: 28555945 DOI: 10.1002/hep.29293] [Citation(s) in RCA: 102] [Impact Index Per Article: 12.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/27/2016] [Revised: 04/14/2017] [Accepted: 05/24/2017] [Indexed: 12/15/2022]
Abstract
UNLABELLED Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disorder characterized by inflammation and fibrosis of the intra- and/or extrahepatic bile ducts. Magnetic resonance imaging (MRI) is a noninvasive imaging modality that can be used to diagnose PSC and detect disease related complications. Quantitative MRI technologies also have the potential to provide valuable prognostic information. Despite the potential of this imaging technology, the clinical application of MRI in the care of PSC patients and imaging standards vary across institutions. Moreover, a unified position statement about the role of MRI in the care of PSC patients, quality imaging standards, and its potential as a research tool is lacking. CONCLUSION Members of the International PSC Study Group and radiologists from North America and Europe have compiled the following position statement to provide guidance regarding the application of MRI in the care of PSC patients, minimum imaging standards, and future areas of research. (Hepatology 2017;66:1675-1688).
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Affiliation(s)
- Christoph Schramm
- 1st Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
| | - John Eaton
- Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN
| | - Kristina I Ringe
- Department of Diagnostic and Interventional Radiology, Hannover Medical School, Hannover, Germany
| | | | - Jin Yamamura
- Department of Diagnostic and Interventional Radiology and Nuclear Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany
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27
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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: 66] [Impact Index Per Article: 8.3] [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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Olpin JD, Sjoberg BP, Stilwill SE, Jensen LE, Rezvani M, Shaaban AM. Beyond the Bowel: Extraintestinal Manifestations of Inflammatory Bowel Disease. Radiographics 2017; 37:1135-1160. [PMID: 28548906 DOI: 10.1148/rg.2017160121] [Citation(s) in RCA: 50] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
Inflammatory bowel disease (IBD) is a chronic, relapsing immune-mediated inflammation of the gastrointestinal tract. IBD includes two major disease entities: Crohn disease and ulcerative colitis. Imaging plays an important role in the diagnosis and surveillance of these complex disorders. Computed tomographic and magnetic resonance enterographic techniques have been refined in recent years to provide a superb means of evaluating the gastrointestinal tract for suspected IBD. Although the intestinal imaging manifestations of IBD have been extensively discussed in the radiology literature, extraintestinal imaging manifestations of IBD have received less attention. Multiple extraintestinal manifestations may be seen in IBD, including those of gastrointestinal (hepatobiliary and pancreatic), genitourinary, musculoskeletal, pulmonary, cardiac, ocular, and dermatologic disorders. Although many associations between IBD and extraintestinal organ systems have been well established, other associations have not been fully elucidated. Some extraintestinal disorders may share a common pathogenesis with IBD. Other extraintestinal disorders may occur as a result of unintended treatment-related complications of IBD. Although extraintestinal disorders within the abdomen and pelvis may be well depicted with cross-sectional enterography, other musculoskeletal and thoracic disorders may be less evident with such examinations and may warrant further investigation with additional imaging examinations or may be readily apparent from the findings at physical examination. Radiologists involved in the interpretation of IBD imaging examinations must be aware of potential extraintestinal manifestations, to provide referring clinicians with an accurate and comprehensive profile of patients with these complex disorders. © RSNA, 2017.
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Affiliation(s)
- Jeffrey D Olpin
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
| | - Brett P Sjoberg
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
| | - Sarah E Stilwill
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
| | - Leif E Jensen
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
| | - Maryam Rezvani
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
| | - Akram M Shaaban
- From the Department of Diagnostic Radiology (J.D.O., S.E.S., L.E.J., M.R., A.M.S.), University of Utah, 30 North 1900 East, #1A71, Salt Lake City, UT 84132; and the Department of Diagnostic Radiology, University of Wisconsin, Madison, Wis (B.P.S.)
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Gidwaney NG, Pawa S, Das KM. Pathogenesis and clinical spectrum of primary sclerosing cholangitis. World J Gastroenterol 2017; 23:2459-2469. [PMID: 28465630 PMCID: PMC5394509 DOI: 10.3748/wjg.v23.i14.2459] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/24/2016] [Revised: 01/21/2017] [Accepted: 03/20/2017] [Indexed: 02/06/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has been documented in the literature since 1867. This disease has a strong predilection for affecting men and can be seen in individuals as young as 2 years of age. PSC has a strong associated with inflammatory bowel disease, more commonly with ulcerative colitis, and is also part of the clinical spectrum of IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association with inflammatory bowel disease. The exact pathogenesis of PSC is not well understood at present, however, is likely a combination of a genetic predisposition with alteration of the molecular structure of the gut. Abnormal serum liver chemistry and presence of certain autoimmune markers are usually the first indicators leading to a diagnosis of PCS, however, these may often be normal in early stages of this disease. The diagnosis is made by cholangiography, which is now considered the gold standard. PSC is a known pre-malignant condition. Such patients have an increased risk of developing cholangiocarcinoma, gallbladder neoplasia, and colon cancer. Many new treatment modalities have emerged in the recent past, including anti-tumor necrosis factor- α and anti-integrins; however, liver transplantation is the only known cure for PSC. Despite past and present research, PSC remains an enigmatic biliary disease with few viable treatment options.
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30
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Restellini S, Chazouillères O, Frossard JL. Hepatic manifestations of inflammatory bowel diseases. Liver Int 2017; 37:475-489. [PMID: 27712010 DOI: 10.1111/liv.13265] [Citation(s) in RCA: 54] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/29/2016] [Accepted: 09/27/2016] [Indexed: 12/18/2022]
Abstract
Inflammatory bowel diseases are associated with various hepatobiliary disorders, reported both in Crohn's disease and ulcerative colitis. They may occur at any moment in the natural course of the disease. The prevalence of liver dysfunction rises from 3% to 50% accordingly to definitions used in different studies. Fatty liver is considered as the most common hepatobiliary complication in inflammatory bowel diseases while primary sclerosing cholangitis is the most specific one. Less frequently, inflammatory bowel diseases-associated hepatobiliary disorders include: autoimmune hepatitis/ primary sclerosing cholangitis overlap syndrome, IgG4-associated cholangiopathy, primary biliary cholangitis, hepatic amyloidosis, granulomatous hepatitis, cholelithiasis, portal vein thrombosis and liver abscess. The spectrum of these manifestations varies according to the type of inflammatory bowel diseases. Treatments of inflammatory bowel diseases may cause liver toxicity, although incidence of serious complications remains low. However, early diagnosis of drug-induced liver injury is of major importance as it affects future clinical management. When facing abnormal liver tests, clinicians should undertake a full diagnostic work-up in order to determine whether the hepatic abnormalities are related to the inflammatory bowel diseases or not. Management of hepatic manifestations in inflammatory bowel diseases usually involves both hepatologists and gastroenterologists because of the complexity of some situations.
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Affiliation(s)
- Sophie Restellini
- Service de Gastroentérologie et Hépatologie, Hôpitaux Universitaires de Genève, Genève, Suisse
| | - Olivier Chazouillères
- Division d'Hépatologie, Centre de Référence des Maladies Inflammatoires des Voies Biliaires, et Université de Sorbonne, UPMC Univ Paris 06, UMR_S 938, CDR Saint-Antoine, AP-HP, Hôpital Saint-Antoine, Paris, France
| | - Jean-Louis Frossard
- Service de Gastroentérologie et Hépatologie, Hôpitaux Universitaires de Genève, Genève, Suisse
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Koyama K, Kubo S, Ueki A, Shimono T, Takemura S, Tanaka S, Kinoshita M, Hamano G, Miki Y. MR imaging and MR cholangiopancreatography of cholangiocarcinoma developing in printing company workers. Jpn J Radiol 2017; 35:233-241. [PMID: 28255646 DOI: 10.1007/s11604-017-0626-y] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/07/2016] [Accepted: 02/19/2017] [Indexed: 12/11/2022]
Abstract
PURPOSE To retrospectively investigate magnetic resonance (MR) imaging findings of occupational cholangiocarcinoma (oCC) occurring among workers in printing companies in Japan, compared to those of non-occupational cholangiocarcinoma (nCC), primary sclerosing cholangitis (PSC), and age-matched normal controls (NORs). MATERIALS AND METHODS Participants comprised 27 consecutive patients (oCC, n = 5; nCC, n = 8; PSC, n = 6; NOR, n = 8) who underwent MR imaging between May 2009 and October 2012. MR imaging was evaluated with respect to tumor characteristics, abnormal MR cholangiographic findings (PSC-like findings), bile duct stricture, and signal changes of the hepatic parenchyma. RESULTS Tumors were detected in all nCCs and four oCCs. Tumors displayed a mass-forming type in all nCCs and two oCCs, and an intraductal growth type in two oCCs. Abnormal cholangiographic findings were detected in all oCCs and PSCs, but not in any nCCs or NORs. All oCCs and seven nCCs showed biliary strictures longer than 1 cm; five PSCs showed biliary strictures shorter than 1 cm. Both intra- and extrahepatic biliary strictures were detected in three PSCs and two oCCs. Peripheral hepatic hyperintensity on T2-weighted imaging was detected in two nCCs, two PSCs, and two oCCs. CONCLUSION These results indicated that MR imaging of oCC showed findings of both PSC and nCC.
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Affiliation(s)
- Koichi Koyama
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka-City, Osaka, 545-8585, Japan.
| | - Shoji Kubo
- Department of Hepato-Biliary-Pancreatic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Ai Ueki
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka-City, Osaka, 545-8585, Japan
| | - Taro Shimono
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka-City, Osaka, 545-8585, Japan
| | - Shigekazu Takemura
- Department of Hepato-Biliary-Pancreatic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Shogo Tanaka
- Department of Hepato-Biliary-Pancreatic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Masahiko Kinoshita
- Department of Hepato-Biliary-Pancreatic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Genya Hamano
- Department of Hepato-Biliary-Pancreatic Surgery, Osaka City University Graduate School of Medicine, Osaka, Japan
| | - Yukio Miki
- Department of Diagnostic and Interventional Radiology, Osaka City University Graduate School of Medicine, 1-4-3 Asahi-machi, Abeno-ku, Osaka-City, Osaka, 545-8585, Japan
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Abstract
PURPOSE OF REVIEW Primary sclerosing cholangitis (PSC) is a rare, idiopathic biliary disease often with an insidious onset, variable disease course, and premature death related to benign and malignant PSC-related sequelae. This review aims to discuss the epidemiology, clinical variants, and natural history of PSC, incorporating data from recent population-based studies. RECENT FINDINGS PSC naturally leads to cirrhosis, cholangiocarcinoma, other hepatobiliary malignancies, dominant strictures, hepatic osteodystrophy, and bacterial cholangitis. The incidence of PSC appears to be increasing, the reasons for which are unclear. The time from diagnosis to liver transplant appears to be longer in more recent studies compared with earlier studies, suggesting a better overall prognosis than previously believed. In addition, with an increasing number of patients undergoing liver transplantation for PSC, the frequency of death because of liver failure has decreased, whereas cancer-related deaths have increased among patients with PSC. SUMMARY PSC is a heterogeneous disease with a variety of clinical outcomes, both fatal and nonfatal. The progression of liver fibrosis in an individual patient is difficult to predict and may vary from a relatively benign, nonprogressive form to a rapidly progressive form with the need for liver transplantation.
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Ni Mhuircheartaigh JM, Lee KS, Curry MP, Pedrosa I, Mortele KJ. Early Peribiliary Hyperenhancement on MRI in Patients with Primary Sclerosing Cholangitis: Significance and Association with the Mayo Risk Score. Abdom Radiol (NY) 2017; 42:152-158. [PMID: 27472938 DOI: 10.1007/s00261-016-0847-z] [Citation(s) in RCA: 23] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/25/2022]
Abstract
The aims of this study are to assess any relationship between peribiliary hyperenhancement on MRI in patients with primary sclerosing cholangitis (PSC) and their Mayo risk score and to assess which timing of peribiliary hyperenhancement correlates best with the Mayo risk score. In this HIPAA-compliant, IRB-approved retrospective study, 101 patients who underwent MRI for known or suspected PSC were identified. Of those, 62 patients (mean age 48 years; 40 males) were diagnosed with PSC by a hepatologist based on findings on MRI, ERCP, and/or liver biopsy, and comprise the final cohort. Data were recorded on whether peribiliary hyperenhancement was present, the post-contrast phase and the extent of involvement. The components to calculate the Mayo risk score were recorded. Statistical analysis was performed using the student T test, Fisher's exact test, and the Kaplan-Meier estimate. Of 62 patients, 41 (66.1%) patients had a low-Mayo risk score (<0), 14 (22.6%) had an intermediate-risk score (≤2 and >0), and 7 (11.3%) had a high-risk score (>2). On MRI, 29 (46.8%) patients demonstrated arterial peribiliary hyperenhancement. Both the presence and extent of peribiliary hyperenhancement showed significant associations with Mayo risk score (p < 0.01). Using the combined end point of liver transplantation or death, there was a statistically significant difference in survival times between those with and those without arterial peribiliary hyperenhancement (p < 0.05). The presence of arterial peribiliary hyperenhancement in patients with PSC on MRI is associated with higher Mayo risk scores and may suggest a poorer prognosis.
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Affiliation(s)
| | - Karen S Lee
- Department of Radiology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA, 02215, USA
| | - Michael P Curry
- Department of Hepatology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA, 02215, USA
| | - Ivan Pedrosa
- Department of Radiology, UT Southwestern, 5323 Harry Hines Blvd, Dallas, TX, 75390, USA
| | - Koenraad J Mortele
- Department of Radiology, Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA, 02215, USA
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de Vries E, Beuers U. Management of cholestatic disease in 2017. Liver Int 2017; 37 Suppl 1:123-129. [PMID: 28052628 DOI: 10.1111/liv.13306] [Citation(s) in RCA: 62] [Impact Index Per Article: 7.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/26/2016] [Accepted: 10/27/2016] [Indexed: 12/13/2022]
Abstract
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic (PBC) or larger intra- and/or extrahepatic (PSC) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified. Ursodeoxycholic acid (UDCA) is the standard treatment of PBC and is used also for other cholestatic conditions including PSC, and it exerts anticholestatic effects at adequate doses. Novel anticholestatic therapeutic options for patients not adequately responding to UDCA are under development or have, like obeticholic acid, already been proven to have efficacy when combined with UDCA in the treatment of PBC. The future role of immunomodulating/immunosuppressive drug regimens must be critically reviewed.
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Affiliation(s)
- Elsemieke de Vries
- Department of Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
| | - Ulrich Beuers
- Department of Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
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Coelho-Prabhu N, Martin JA. Dilation of Strictures in Patients with Inflammatory Bowel Disease: Who, When and How. Gastrointest Endosc Clin N Am 2016; 26:739-59. [PMID: 27633600 DOI: 10.1016/j.giec.2016.06.011] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Stricture formation occurs in up to 40% of patients with inflammatory bowel disease (IBD). Patients are often symptomatic, resulting in significant morbidity, hospitalizations, and loss of productivity. Strictures can be managed endoscopically in addition to traditional surgical management (sphincteroplasty or resection of the affected bowel segments). About 3% to 5% patients with IBD develop primary sclerosing cholangitis (PSC), which results in stricture formation in the biliary tree, managed for the most part by endoscopic therapies. In this article, we discuss endoscopic management of strictures both in the alimentary tract and biliary tree in patients with IBD and/or PSC.
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Affiliation(s)
- Nayantara Coelho-Prabhu
- Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA
| | - John A Martin
- Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA.
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Abstract
The diagnostics of diffuse liver disease traditionally rely on liver biopsies and histopathological analysis of tissue specimens. However, a liver biopsy is invasive and carries some non-negligible risks, especially for patients with decreased liver function and those requiring repeated follow-up examinations. Over the last decades, magnetic resonance imaging (MRI) has developed into a valuable tool for the non-invasive characterization of focal liver lesions and diseases of the bile ducts. Recently, several MRI methods have been developed and clinically evaluated that also allow the diagnostics and staging of diffuse liver diseases, e.g. non-alcoholic fatty liver disease, hepatitis, hepatic fibrosis, liver cirrhosis, hemochromatosis and hemosiderosis. The sequelae of diffuse liver diseases, such as a decreased liver functional reserve or portal hypertension, can also be detected and quantified by modern MRI methods. This article provides the reader with the basic principles of functional MRI of the liver and discusses the importance in a clinical context.
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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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Hadizadeh M, Abedi SH, Malekpour H, Radinnia E, Jabbehdari S, Padashi M, Zali MR, Mohammad Alizadeh AH. Prevalence of inflammatory bowel disease among patients with primary sclerosing cholangitis in Iran. Arab J Gastroenterol 2016; 17:17-9. [PMID: 27032493 DOI: 10.1016/j.ajg.2015.09.004] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/03/2014] [Revised: 02/27/2015] [Accepted: 07/09/2015] [Indexed: 02/07/2023]
Abstract
BACKGROUND AND STUDY AIMS In Iran, the epidemiology of primary sclerosing cholangitis (PSC) and its association with inflammatory bowel disease (IBD) have not been studied thoroughly. This study investigates the epidemiology and prevalence of IBD among patients with PSC. PATIENTS AND METHODS A retrospective study of 154 patients with PSC was conducted. The demographic and clinical data were collected, and the variables were analysed in the following two patient groups: patients with both PSC and IBD, and patients with PSC and without IBD. RESULTS A total of 154 patients with a mean age of 40.3years (range 20-81years) were included, of whom 57 (37%) were female and 97 (63%) male. Ninety-six patients (62.3%) were diagnosed with IBD, 92 (59.7%) with ulcerative colitis, and four (2.6%) with Crohn's disease. In this study, elevated alkaline phosphatase levels were found in 90.8% of patients. The intra-hepatic duct (IHD) and extrahepatic duct (EHD) were involved in 70.5% of patients, isolated intrahepatic bile duct in 24.4%, and isolated extrahepatic duct in 3.8%. Small-duct PSC (normal results of imaging and PSC proved by histology of liver biopsy) was observed only in 1.3% of patients. CONCLUSION PSC has become increasingly diagnosed in Iran. This is possibly because of better diagnostics and the increasing prevalence of IBD in this country. For diagnosing PSC and identifying the presence of IHD strictures, the results of both magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) showed good interobserver agreement, but the ERCP results could be used to evaluate the presence and severity of EHD strictures.
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Affiliation(s)
- Mohammad Hadizadeh
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Seyed H Abedi
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Habib Malekpour
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Ebrahim Radinnia
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Sayena Jabbehdari
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Maryam Padashi
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Mohammad R Zali
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran
| | - Amir H Mohammad Alizadeh
- Research Center for Gastroenterology and Liver Diseases, Shahid Beheshti University of Medical Sciences, Iran.
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Ferreira R, Loureiro R, Nunes N, Santos AA, Maio R, Cravo M, Duarte MA. Role of endoscopic retrograde cholangiopancreatography in the management of benign biliary strictures: What’s new? World J Gastrointest Endosc 2016; 8:220-231. [PMID: 26962404 PMCID: PMC4766255 DOI: 10.4253/wjge.v8.i4.220] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/02/2015] [Revised: 08/18/2015] [Accepted: 12/15/2015] [Indexed: 02/05/2023] Open
Abstract
Benign biliary strictures comprise a heterogeneous group of diseases. The most common strictures amenable to endoscopic treatment are post-cholecystectomy, post-liver transplantation, related to primary sclerosing cholangitis and to chronic pancreatitis. Endoscopic treatment of benign biliary strictures is widely used as first line therapy, since it is effective, safe, noninvasive and repeatable. Endoscopic techniques currently used are dilation, multiple plastic stents insertion and fully covered self-expandable metal stents. The main indication for dilation alone is primary sclerosing cholangitis related strictures. In the vast majority of the remaining cases, temporary placement of multiple plastic stents with/without dilation is considered the treatment of choice. Although this approach is effective, it requires multiple endoscopic sessions due to the short duration of stent patency. Fully covered self-expandable metal stents appear as a good alternative to plastic stents, since they have an increased radial diameter, longer stent patency, easier insertion technique and similar efficacy. Recent advances in endoscopic technique and various devices have allowed successful treatment in most cases. The development of novel endoscopic techniques and devices is still ongoing.
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Nayagam JS, Pereira SP, Devlin J, Harrison PM, Joshi D. Controversies in the management of primary sclerosing cholangitis. World J Hepatol 2016; 8:265-272. [PMID: 26925200 PMCID: PMC4757649 DOI: 10.4254/wjh.v8.i5.265] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/01/2015] [Revised: 10/13/2015] [Accepted: 01/22/2016] [Indexed: 02/06/2023] Open
Abstract
Primary sclerosing cholangitis (PSC) remains a rare but significant disease, which affects mainly young males in association with inflammatory bowel disease. There have been few advances in the understanding of the pathogenesis of the condition and no therapeutics with proven mortality benefit aside from liver transplantation. There remain areas of controversy in the management of PSC which include the differentiation from other cholangiopathies, in particular immunoglobulin G4 related sclerosing cholangitis, the management of dominant biliary strictures, and the role of ursodeoxycholic acid. In addition, the timing of liver transplantation in PSC remains difficult to predict with standard liver severity scores. In this review, we address these controversies and highlight the latest evidence base in the management of PSC.
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Abstract
Sclerosing cholangitis in pediatric age is a severe disease, often associated with inflammatory bowel disease. It recognizes different etiologies. Management and prognosis depend on the underlying cause. A high proportion of patients have autoimmune features similar to those of autoimmune hepatitis and respond biochemically to immunosuppression, although bile duct disease progresses in half of them leading to liver transplant. The disease can recur after transplant. Severity of liver disease and risk of recurrence after transplant are linked to the severity of bowel disease.
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Affiliation(s)
- Giorgina Mieli-Vergani
- Paediatric Liver, GI and Nutrition Centre, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
| | - Diego Vergani
- Institute of Liver Studies, King's College Hospital, Denmark Hill, London SE5 9RS, UK
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42
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Abstract
Research related to primary sclerosing cholangitis (PSC) has since 1980 been a major activity at the Oslo University Hospital Rikshospitalet. The purpose of this publication is to describe the development of this research, the impact of this research on the clinical handling of the patients, and finally to describe what we believe are the most urgent, remaining problems to be solved. During the early years, our research dealt primarily with clinical aspects of the disease. The concomitant inflammatory bowel disease (IBD) seen in most patients with PSC was a major interest and we also started looking into genetic associations of PSC. Prognosis, malignancy development and treatment with special emphasis on transplantation have later been dealt with. These activities has had impact on several aspects of PSC management; when and how to diagnose PSC and variant forms of PSC, how to handle IBD in PSC and how to deal with the increased rate of malignancy? The problems remaining to be solved are many. What is the role of the gut and the gut microbiota in the development of PSC? Do the PSC patients have an underlying disturbance in the bile homeostasis? And how does the characteristic type of fibrosis in PSC develop? The genetic studies have supported a role for the adaptive immune system in the disease development, but how should this be dealt with? Importantly, the development of malignancy in PSC is still not understood, and we lack appropriate medical treatment for our patients.
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Affiliation(s)
- Erik Schrumpf
- Norwegian PSC research center, Department of Transplantation Medicine, Division of Cancer Medicine, Surgery and Transplantation, Oslo University Hospital Rikshospitalet , Oslo , Norway
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43
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Lindor KD, Kowdley KV, Harrison ME. ACG Clinical Guideline: Primary Sclerosing Cholangitis. Am J Gastroenterol 2015; 110:646-59; quiz 660. [PMID: 25869391 DOI: 10.1038/ajg.2015.112] [Citation(s) in RCA: 330] [Impact Index Per Article: 33.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2014] [Accepted: 03/02/2015] [Indexed: 12/11/2022]
Abstract
Primary sclerosing cholangitis is a chronic cholestatic liver disease that can shorten life and may require liver transplantation. The cause is unknown, although it is commonly associated with colitis. There is no approved or proven therapy, although ursodeoxycholic acid is used by many on an empiric basis. Complications including portal hypertension, fat-soluble vitamin deficiency, metabolic bone diseases, and development of cancers of the bile duct or colon can occur.
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Affiliation(s)
- Keith D Lindor
- 1] College of Health Solutions, Arizona State University, Phoenix, Arizona, USA [2] Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, Arizona, USA
| | - Kris V Kowdley
- Liver Care Network and Organ Care Research, Swedish Medical Center, Seattle, Washington, USA
| | - M Edwyn Harrison
- Division of Gastroenterology and Hepatology, Mayo Clinic, Phoenix, Arizona, USA
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Carrasco-Avino G, Schiano TD, Ward SC, Thung SN, Fiel MI. Primary sclerosing cholangitis: detailed histologic assessment and integration using bioinformatics highlights arterial fibrointimal hyperplasia as a novel feature. Am J Clin Pathol 2015; 143:505-13. [PMID: 25780002 DOI: 10.1309/ajcpvkfviprbxqr2] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
OBJECTIVES Liver biopsy diagnosis of primary sclerosing cholangitis (PSC) is difficult. We performed a detailed histologic analysis of PSC cases using novel bioinformatics analysis to identify histologic features that may be useful in its diagnosis. METHODS PSC liver explants were examined and compared with primary biliary cirrhosis and hepatitis C explants to act as controls. Demographic, macroscopic, and histologic variables were analyzed using both conventional statistics and an integrative bioinformatics approach, significance analysis of microarrays (SAM), and hierarchical clustering analysis (HCA). RESULTS The PSC group was younger and had distinctive PSC features, including bile duct scars, onion-skin fibrosis, and arterial fibrointimal hyperplasia. SAM allowed the integration of variables by comparing PSC and control groups, whereas HCA was able to correctly categorize each group. CONCLUSIONS This study demonstrates characteristic PSC histology as well as arterial hyperplasia to be distinctive features that may aid in PSC diagnosis and be confirmed by bioinformatics.
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Affiliation(s)
| | - Thomas D. Schiano
- Division of Liver Diseases and Recanati-Miller Transplant Institute, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Stephen C. Ward
- Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY
| | - Swan N. Thung
- Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY
| | - M. Isabel Fiel
- Department of Pathology, Icahn School of Medicine at Mount Sinai, New York, NY
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Chathadi KV, Chandrasekhara V, Acosta RD, Decker GA, Early DS, Eloubeidi MA, Evans JA, Faulx AL, Fanelli RD, Fisher DA, Foley K, Fonkalsrud L, Hwang JH, Jue TL, Khashab MA, Lightdale JR, Muthusamy VR, Pasha SF, Saltzman JR, Sharaf R, Shaukat A, Shergill AK, Wang A, Cash BD, DeWitt JM. The role of ERCP in benign diseases of the biliary tract. Gastrointest Endosc 2015; 81:795-803. [PMID: 25665931 DOI: 10.1016/j.gie.2014.11.019] [Citation(s) in RCA: 96] [Impact Index Per Article: 9.6] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2014] [Accepted: 11/17/2014] [Indexed: 12/29/2022]
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Navaneethan U, Jegadeesan R, Nayak S, Lourdusamy V, Sanaka MR, Vargo JJ, Parsi MA. ERCP-related adverse events in patients with primary sclerosing cholangitis. Gastrointest Endosc 2015; 81:410-419. [PMID: 25085336 DOI: 10.1016/j.gie.2014.06.030] [Citation(s) in RCA: 43] [Impact Index Per Article: 4.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/12/2014] [Accepted: 06/11/2014] [Indexed: 02/08/2023]
Abstract
BACKGROUND ERCP is frequently used in patients with primary sclerosing cholangitis (PSC) for cancer surveillance and treatment of dominant strictures. OBJECTIVE To evaluate the prevalence and risk factors for ERCP-related adverse events in patients with PSC. DESIGN Retrospective analysis of ERCPs performed from 1998 to 2012. SETTING Referral center. PATIENTS A total of 294 consecutive patients with PSC who underwent a total of 657 ERCPs. INTERVENTIONS ERCP. MAIN OUTCOME MEASUREMENTS ERCP-related adverse events and predictive factors were determined by univariate and multivariate analyses. RESULTS ERCP use in patients with PSC showed a significant increase during the second half of the study period (2006-2012) compared with the first half (1998-2005) (437 vs 220 procedures; P = .04). Primary cannulation was successful in 634 procedures (96.6%) or in 271 of 294 patients (92.2%). Access to the bile duct was achieved with a needle-knife in 19 procedures (2.9%), whereas ERCP was unsuccessful in 4 of 657 procedures (0.6%), and successful percutaneous drainage was performed. Post-ERCP pancreatitis (PEP) was diagnosed in 8 (1.2%), cholangitis in 16 (2.4%), and bleeding in 4 (0.7%) procedures. Overall, risk of any adverse event was 28 of 657 (4.3%) procedures. On multivariate analysis, performing biliary sphincterotomy (odds ratio [OR] 5.04; 95% confidence interval [CI], 2.01-12.60; P = .001) and passage of a guidewire into the pancreatic duct (OR 4.54; 95% CI, 1.44-14.30; P = .010) were independently associated with an increased risk of any adverse event. LIMITATIONS Retrospective study. CONCLUSION Cholangitis appears to be the most common adverse event despite intraprocedural antibiotic use. There was a low risk of adverse events in patients with PSC undergoing ERCP.
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Affiliation(s)
- Udayakumar Navaneethan
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Ramprasad Jegadeesan
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Shishira Nayak
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Vennisvasanth Lourdusamy
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Madhusudhan R Sanaka
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - John J Vargo
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
| | - Mansour A Parsi
- Department of Gastroenterology, Digestive Disease Institute, Cleveland Clinic, Cleveland, Ohio, USA
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Thosani N, Banerjee S. Endoscopic retrograde cholangiopancreatography for primary sclerosing cholangitis. Clin Liver Dis 2014; 18:899-911. [PMID: 25438290 DOI: 10.1016/j.cld.2014.07.013] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
Although there are no randomized, controlled trials evaluating the efficacy of endoscopic retrograde cholangiography (ERC) in primary sclerosing cholangitis (PSC) patients, substantial indirect evidence supports the effectiveness of ERC in symptomatic PSC patients with a dominant stricture. Currently, cumulative evidence supports the role of ERC with endoscopic dilation with or without additional short-term stent placement for symptomatic PSC patients with a dominant stricture. Differentiating benign dominant strictures from cholangiocarcinoma (CCA) remains difficult; however, newer endoscopic techniques and advanced cytologic techniques are likely to improve sensitivity for the diagnosis of CCA over that achieved by traditional cytology brushing alone.
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Affiliation(s)
- Nirav Thosani
- Division of Gastroenterology and Hepatology, Stanford University School of Medicine, 300 Pasteur Drive, MC: 5244, Stanford, CA 94305, USA
| | - Subhas Banerjee
- Division of Gastroenterology and Hepatology, Stanford University School of Medicine, 300 Pasteur Drive, MC: 5244, Stanford, CA 94305, USA.
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Abstract
Although ultrasound, computed tomography, and cholescintigraphy play essential roles in the evaluation of suspected biliary abnormalities, magnetic resonance (MR) imaging and MR cholangiopancreatography can be used to evaluate inconclusive findings and provide a comprehensive noninvasive assessment of the biliary tract and gallbladder. This article reviews standard MR and MR cholangiopancreatography techniques, clinical applications, and pitfalls. Normal biliary anatomy and variants are discussed, particularly as they pertain to preoperative planning. A spectrum of benign and malignant biliary processes is reviewed, emphasizing MR findings that aid in characterization.
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Ruiz A, Lemoinne S, Carrat F, Corpechot C, Chazouillères O, Arrivé L. Radiologic course of primary sclerosing cholangitis: assessment by three-dimensional magnetic resonance cholangiography and predictive features of progression. Hepatology 2014; 59:242-50. [PMID: 23857427 DOI: 10.1002/hep.26620] [Citation(s) in RCA: 100] [Impact Index Per Article: 9.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/09/2013] [Accepted: 07/01/2013] [Indexed: 01/10/2023]
Abstract
UNLABELLED Magnetic resonance imaging (MRI) with magnetic resonance cholangiography (MRC) has become the radiologic standard of reference for diagnosis of primary sclerosing cholangitis (PSC). However, natural history of radiologic features of PSC is poorly known. In the current study, we aimed at analyzing the course of PSC using three-dimensional (3D) MRC and liver MRI to find predictive radiologic features of progression. PSC patients, followed up in our center, with at least two 3D MRCs performed in at least a 1-year interval, were retrospectively reviewed. We built an interpretation standard model to score precisely bile ducts and liver parenchyma features. The primary endpoint was overall radiologic course, including worsening, improvement, or stabilization. Radiologic features were analyzed by logistic regression. We reviewed 289 MRIs from 64 patients upon a mean radiologic follow-up of 4 years (range, 1-9). Radiologic features worsened in 37 patients (58%) and stabilized in 27 (42%); no patient showed improvement. Multivariate analysis resulted in two MRI progression risk scores, based on the combination of predictive radiologic features (score without gadolinium administration = 1 × dilatation of intrahepatic bile ducts + 2 × dysmorphy + 1 × portal hypertension; score with gadolinium administration = 1 × dysmorphy + 1 × parenchymal enhancement heterogeneity). These scores were associated with radiologic progression, with an area under the curve of 80 and 83% ± 4%. CONCLUSION A majority of PSC patients develop radiologic aggravation upon MRI over 4 years. Two simple scores can predict radiologic progression.
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Affiliation(s)
- Ana Ruiz
- APHP, Service de radiologie, Hôpital Saint Antoine, Paris, France and Université Pierre et Marie Curie (UPMC, Paris VI), Paris, France
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Sugita R. Magnetic resonance evaluations of biliary malignancy and condition at high-risk for biliary malignancy: Current status. World J Hepatol 2013; 5:654-665. [PMID: 24432183 PMCID: PMC3888665 DOI: 10.4254/wjh.v5.i12.654] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/25/2013] [Revised: 07/26/2013] [Accepted: 12/11/2013] [Indexed: 02/06/2023] Open
Abstract
Tumors of the biliary tree are relatively rare; but their incidence is rising worldwide. There are several known risk factors for bile duct cancers, and these are seem to be associated with chronic inflammation of the biliary epithelium. Herein, 2 risk factors have been discussed, primary sclerosing cholangitis and reflux of pancreatic juice into the bile duct, as seen in such as an abnormal union of the pancreatic-biliary junction because magnetic resonance imaging (MRI) is used widely and effectively in the diagnosis of these diseases. When biliary disease is suspected, MRI can often help differentiate between benignity and malignancy, stage tumors, select surgical candidates and guide surgical planning. MRI has many advantages over other modalities. Therefore, MRI is a reliable noninvasive imaging tool for diagnosis and pre-surgical evaluation of bile duct tumors. Nowadays remarkable technical advances in magnetic resonance technology have expanded the clinical applications of MRI in case of biliary diseases. In this article, it is also discussed how recent developments in MRI contributes to the diagnosis of the bile duct cancer and the evaluation of patients with risk factors affecting bile duct cancer.
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