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Digital single-operator video cholangioscopy improves endoscopic management in patients with primary sclerosing cholangitis-a retrospective observational study

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World Journal of Gastroenterology 2022年第20期28卷 2201-2213页

作者： Arne Bokemeyer Frank Lenze Viorelia Stoica Timur Selcuk Sensoy Iyad Kabar Hartmut Schmidt Hansjoerg Ullerich Department of Medicine B(Gastroenterology HepatologyEndocrinologyClinical Infectiology)University Hospital MuensterMuenster 48149Germany Department of Gastroenterology Hepatology and Transplant MedicineUniversity Hospital EssenEssen 45147Germany

BACKGROUND Patients with primary sclerosing cholangitis(PSC)are at a high risk of developing cholestatic liver disease and biliary cancer,and endoscopy is crucial for the complex management of these patients.AIM To clarify the utility of recently introduced digital single-operator video cholangioscopy(SOVC)for the endoscopic management of PSC patients.METHODS In this observational study,all patients with a history of PSC and in whom digital SOVC(using the SpyGlass DS System)was performed between 2015 and 2019 were included and retrospectively analysed.Examinations were performed at a tertiary referral centre in Germany.In total,46 SOVCs performed in 38 patients with a history of PSC were identified.The primary endpoint was the evaluation of dominant biliary strictures using digital SOVC,and the secondary endpoints were the performance of selective guidewire passage across biliary strictures and the diagnosis and treatment of biliary stone disease in PSC patients.RESULTS The 22 of 38 patients had a dominant biliary stricture(57.9%).In 4 of these 22 patients,a cholangiocellular carcinoma was diagnosed within the stricture(18.2%).Diagnostic evaluation of dominant biliary strictures using optical signs showed a sensitivity of 75%and a specificity of 94.4%to detect malignant strictures,whereas SOVC-guided biopsies to gain tissue for histopathological analysis showed a sensitivity of 50%and a specificity of 100%.In 13%of examinations,SOVC was helpful for guidewire passage across biliary strictures that could not be passed by conventional methods(technical success rate 100%).Biliary stone disease was observed in 17.4%of examinations;of these,in 37.5%of examinations,biliary stones could only be visualized by SOVC and not by standard fluoroscopy.Biliary stone treatment was successful in all cases(100%);25%required SOVC-assisted electrohydraulic lithotripsy.Complications,such as postinterventional cholangitis and pancreatitis,occurred in 13%of examinations;however,no procedure-associated mortality occurred.CONCLUSION Digit

关键词： Cholangitis sclerosing Biliary tract diseases Biliary strictures Endoscopy Gastrointestinal Cholangioscopy Digital single-operator video cholangioscopy

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Recurrence and rejection in liver transplantation for primary sclerosing cholangitis

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World Journal of Gastroenterology 2012年第1期18卷 1-15页

作者： Bjarte Fosby Tom H Karlsen Espen Melum Department of Transplantation Surgery Clinic for Specialized Medicine and Surgery Oslo University Hospital Norwegian Primary sclerosing cholangitis Research Center Clinic for Specialized Medicine and Surgery Oslo University Hospital Research Institute for Internal Medicine Oslo University Hospital

Primary sclerosing cholangitis (PSC) is a chronic progressive inflammatory disease affecting the bile ducts, leading to f ibrosis and eventually cirrhosis in most patients. Its etiology is unknown and so far no effective medical therapy is available. Liver transplantation (LTX) is the only curative treatment and at present PSC is the main indication for LTX in the Scandinavian countries. Close to half of the PSC patients experience one or more episodes of acute cellular rejection (ACR) following transplantation and approximately 1/5 of the transplanted patients develop recurrent disease in the graft. In addition, some reports indicate that ACR early after LTX for PSC can infl uence the risk for recurrent disease. For these important post-transplantation entities affecting PSC patients, we have reviewed the current literature on epidemiology, pathogenesis, treatment and the possible infl uence of rejection on the risk of recurrent disease in the allograft.

关键词： Liver transplantation Primary sclerosing cholangitis Rejection Recurrence

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Role of colectomy in preventing recurrent primary sclerosing cholangitis in liver transplant recipients

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World Journal of Gastroenterology 2018年第28期24卷 3171-3180页

作者： Bettina M Buchholz Panagis M Lykoudis Reena Ravikumar Joerg M Pollok Giuseppe K Fusai Department of HPB Surgery and Liver Transplantation Royal Free Hospital London

AIM To study the published evidence on the impact of colectomy in preventing recurrent primary sclerosing cholangitis(r PSC). METHODS An unrestricted systematic literature search in Pub Med, EMBASE, Medline Ovid SP, ISI Web of Science, Lista(EBSCO) and the Cochrane library was performed on clinical studies investigating colectomy in liver transplantation(LT) recipients with and without r PSC in the liver allograft. Study quality was evaluated according to a modification of the methodological index for non-randomized studies(MINORS) criteria. Primary endpoints were the impact of presence, timing and type of colectomy on r PSC. Overall presence of inflammatory bowel disease(IBD), time of IBD diagnosis, posttransplant IBD and immunosuppressive regimen were investigated as secondary outcome.RESULTS The literature search yielded a total of 180 publications. No randomized controlled trial was identified. Six retrospective studies met the inclusion criteria of which 5 studies were graded as high quality articles. Reporting of IBD was heterogenous but in four publications, either inflammatory bowel disease, ulcerative colitis or in particular active colitis post-LT significantly increased the risk of r PSC. The presence of an intact(i.e., retained) colon at LT was identified as risk factor for r PSC in two of the high quality studies while four studies found no effect. Type of colectomy was not associated with r PSC but this endpoint was underreported(only in 33% of included studies).Neither tacrolimus nor cyclosporine A yielded a significant benefit in disease recurrence of primary sclerosing cholangitis(PSC).CONCLUSION The data favours a protective role of pre-/peri-LT colectomy in r PSC but the current evidence is not strong enough to recommend routine colectomy for r PSC prevention.

关键词： Recurrent primary sclerosing cholangitis Risk factor Ulcerative colitis Colectomy Liver transplantation

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Evolving role of magnetic resonance techniques in primary sclerosing cholangitis

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World Journal of Gastroenterology 2019年第6期25卷 644-658页

作者： Emmanuel A Selvaraj Emma L Culver Helen Bungay Adam Bailey Roger W Chapman Michael Pavlides Oxford Centre for Clinical Magnetic Resonance Research Division of Cardiovascular Medicine Radcliffe Department of Medicine University of Oxford John Radcliffe Hospital Translational Gastroenterology Unit Nuffield Department of Medicine University of OxfordJohn Radcliffe Hospital Oxford NIHR Biomedical Research Centre Oxford University Hospitals NHS Foundation Trust and the University of Oxford Department of Radiology John Radcliffe Hospital

Development of non-invasive methods to risk-stratify patients and predict clinical endpoints have been identified as one of the key research priorities in primary sclerosing cholangitis(PSC). In addition to serum and histological biomarkers, there has been much recent interest in developing imaging biomarkers that can predict disease course and clinical outcomes in PSC.Magnetic resonance imaging/magnetic resonance cholangiopancreatography(MRI/MRCP) continue to play a central role in the diagnosis and follow-up of PSC patients. Magnetic resonance(MR) techniques have undergone significant advancement over the last three decades both in MR data acquisition and interpretation. The progression from a qualitative to quantitative approach in MR acquisition techniques and data interpretation, offers the opportunity for the development of objective and reproducible imaging biomarkers that can potentially be incorporated as an additional endpoint in clinical trials. This review article will discuss how the role of MR techniques have evolved over the last three decades from emerging as an alternative diagnostic tool to endoscopic retrograde cholangiopancreatography, to being instrumental in the ongoing search for imaging biomarker of disease stage, progression and prognosis in PSC.

关键词： Primary sclerosing cholangitis Magnetic resonance imaging Magnetic resonance cholangiopancreatography Magnetic resonance elastography Diffusion magnetic resonance imaging Endoscopic retrograde cholangiopancreatography

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World Journal of Gastroenterology 2014年第14期20卷 3986-4000页

作者： Bjarte Fosby Sigrid Nss Johannes R Hov James Traherne Kirsten M Boberg John Trowsdale Aksel Foss Pl-Dag Line Andre Franke Espen Melum Helge Scott Tom H Karlsen Norwegian PSC Research Center Division of CancerSurgery and TransplantationOslo University Hospital Institute of Clinical Medicine Faculty of MedicineUniversity of Oslo KG Jebsen Inflammation Research Centre Research Institute of Internal MedicineOslo University Hospital Department of Transplantation Medicine Division of CancerSurgery and TransplantationOslo University Hospital Division of Immunology Department of PathologyUniversity of Cambridge and Cambridge Institute for Medical ResearchUniversity of Cambridge Institute of Clinical Molecular Biology Christian-Albrechts-University Department of Pathology Divisoin of Diagnostics and InterventionInstitute of PathologyOslo University Hospital

AIM:To investigate influence of human leukocyte antigen(HLA)and killer immunoglobuline-like receptor(KIR)genotypes on risks of acute rejection(AR)after liver transplantation(LTX).METHODS:In this retrospective study we included143 adult donor-recipient pairs with a minimum of 6mo follow-up after LTX for whom DNA was available from both donor and recipients.Clinical data,all early complications including episodes and severity of AR and graft/patient survival were registered.The diagnosis of AR was based on clinical,biochemical and histological criteria.All suspected episodes of AR were biopsy confirmed.Key classical HLA loci(HLA-A,HLA-B,HLA-C and HLA-DRB1)were genotyped using Sanger sequencing.16 KIR genes were genotyped using a novel real time PCR approach which allows for determination of the diploid copy number of each KIR gene.Immunohistochemical staining for T(CD3),B(CD20)and natural killer(NK)cells(CD56 and CD57)were performed on liver biopsies from 3 different patient groups[primary sclerosing cholangitis(PSC),primary biliary cirrhosis and non-autoimmune liver disease],10 in each group,with similar grade of AR.RESULTS:Fourty-four(31%)patients were transplanted on the basis of PSC,40%of them had AR vs 24%in the non-PSC group(P=0.04).No significant impact of donor-recipient matching for HLA and KIR genotypes was detected.In the overall recipient population an increased risk of AR was detected for HLA-B*08(P=0.002,OR=2.5;95%CI:1.4-4.6),HLA-C*07(P=0.001,OR=2.4;95%CI:1.4-4.0)and HLA-DRB1*03(P=0.03,OR=1.9;95%CI:1.0-3.3)and a decreased risk for HLA-DRB1*04(P=0.001,OR=0.2;95%CI:0.1-0.5).For HLA-B*08,HLA-C*07 and DRB1*04 the associations remained evident in a subgroup analysis of non-PSC recipients(P=0.04,P=0.003 and P=0.02,respectively).In PSC recipients corresponding P values were 0.002,0.17 and 0.01 for HLA-B*08,HLA-C*07and DRB1*04,respectively.A dosage effect of AR prevalence according to the PSC associated HLA alleles was also notable in the total recipient population.For HLA-B*08 the frequency of AR was 56%in H

关键词： Liver transplantation Primary sclerosing cholangit

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sclerosing cholangitis secondary to bleomycin-iodinated embolization for liver hemangioma

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World Journal of Gastroenterology 2014年第46期20卷 17680-17685页

作者： Shuo Jin Xiao-Ju Shi Xiao-Dong Sun Si-Yuan Wang Guang-Yi Wang Department of Hepatobiliary and Pancreatic Surgery the First Norman Bethune Hospital Affiliated to Jilin University Department of Surgery Intensive Care Unit Beijing Chao-yang Hospital Affiliated to Capital Medical University

sclerosing cholangitis(SC)is a rarely reported morbidity secondary to transcatheter arterial chemoembolization(TACE)with bleomycin-iodinated oil(BIO)for liver cavernous hemangioma(LCH).This report retrospectively evaluated the diagnostic and therapeutic course of a patient with LDH who presented obstructive jaundice 6 years after TACE with BIO.Preoperative imaging identified a suspected malignant biliary stricture located at the convergence of the left and right hepatic ducts.Operative exploration demonstrated a full-thickness sclerosis of the hilar bile duct with right hepatic duct stricture and right lobe atrophy.Radical hepatic hilar resection with right-side hemihepatectomy and Roux-en-Y hepaticojejunostomy was performed because hilar cancer could not be excluded on frozen biopsy.Pathological results showed chronic pyogenic inflammation of the common and right hepatic ducts with SC in the portal area.Secondary SC is a long-term complication that may occur in LCH patients after TACE with BIO and must be differentiated from hilar malignancy.Hepatic duct plasty is a definitive but technically challenging treatment modality for secondary SC.

关键词： sclerosing cholangitis Secondary Transcatheter art

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Role of endoscopy in the management of primary sclerosing cholangitis

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World Journal of Gastrointestinal Endoscopy 2019年第2期11卷 84-94页

作者： Neil Bharat Marya James H Tabibian Vatche and Tamar Manoukian Division of Digestive Diseases UCLA Gastroenterology Fellowship Training Program Division of Gastroenterology Department of Medicine Olive View-UCLA Medical Center

Primary sclerosing cholangitis(PSC) is a rare but prominent fibroinflammatory cholangiopathy which can affect individuals of essentially any age. It carries a median survival of 15-20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent risk for biliary tract and other complications, and the paucity of effective pharmacotherapies, endoscopy plays a major role in the care of many patients with this disorder. In this review, we discuss the endoscopic management of PSC, including established and evolving approaches to the diagnosis and treatment of its benign as well as malignant sequelae. Owing to the rarity of PSC and dearth of high-quality evidence, we propose pragmatic approaches based on both currently available data and expert opinion.

关键词： Bile duct diseases Cholangiocarcinoma Inflammatory bowel disease Endoscopic retrograde cholangiopancreatography Biopsy Primary sclerosing cholangitis

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Treatment options for spontaneous and postoperative sclerosing mesenteritis

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World Journal of Gastrointestinal Surgery 2016年第11期8卷 761-765页

作者： Jennifer Klasen Ulrich Güller Brigitte Muff Daniel Candinas Christian A Seiler René Fahrner Department of Surgery Kantonsspital Frauenfeld Spital Thurgau AG CH-8500 Frauenfeld Switzerland Division of Medical Oncology and Hematology Kantonsspital St. Gallen CH-9007 St. Gallen Switzerland Division of General Surgery Spital BülachCH-8180 Bülach Switzerland Division of Visceral Surgery and Medicine University Hospital Bern CH-3010 BernSwitzerland Division of General Visceral and Vascular Surgery University Hospital Jena D-07740 Jena Germany

sclerosing mesenteritis is a rare pathology with only a few described cases in the literature. The etiology is unclear; however, several potential triggers, including abdominal surgery and abdominal trauma, have been discussed. The pathology includes a benign acute or chronic inflammatory process affecting the adipose tissue of the mesenterium. Despite it being a rare disease, sclerosing mesenteritis is an important differential diagnosis in patients after abdominal surgery or patients presenting spontaneously with signs of acute inflammation and abdominal pain. We present here three cases with sclerosing mesenteritis. In two cases, sclerosing mesenteritis occurred postoperatively after abdominal surgery. One patient was treated because of abdominal pain and specific radiological signs revealing spontaneous manifestation of sclerosing mesenteritis. So far there are no distinct treatment algorithms, so the patients were treated differently, including steroids, antibiotics and watchful waiting. In addition, we reviewed the current literature on treatment options for this rare disease.

关键词： sclerosing mesenteritis Abdominal pain Inflammation Surgery Immunosuppression

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Primary esophageal sclerosing mucoepidermoid carcinoma with “tissue eosinophilia”

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World Journal of Gastroenterology 2014年第22期20卷 7055-7060页

作者： Suman Mewa Kinoo Kapil Maharaj Bhugwan Singh Michelle Govender Pratistadevi Kanaye Ramdial Department of Surgery Nelson R Mandela School of MedicineUniversity of Kwazulu NatalDurban 4013South Africa Department of Anatomical Pathology School of Laboratory Medicine and Medical SciencesUniversity of Kwazulu Natal and National Health Laboratory ServiceDurban 4013South Africa

Mucoepidermoid carcinoma(MEC)is a rare primary esophageal malignancy.It is characterized by poor clinical recognition,pre-operative diagnostic challenges and a lack of standardized therapeutic guidelines.We report the clinicopathological features of a hitherto unreported variant of esophageal MEC,sclerosing MEC with"tissue eosinophilia",in a mid-esophageal location in a 51-year-old female.The diagnosis of the initial biopsy was challenging,because of the small size,poor orientation and inadequate representation of the MEC components.Recognition of the resectability of the tumor prompted surgical resection and enabled a demonstration of the low grade foci containing intermediate cells,mucin pools and the hitherto undescribed presence of stromal sclerosis and tissue eosinophils in esophageal MEC.Heightened clinicopathological awareness of esophageal MEC facilitated a definitive diagnosis and patient management.Increased recognition andglobal documentation of esophageal sclerosing MEC with"tissue eosinophilia"is necessary to improve the understanding and diagnosis of this malignancy in this location and to improve management guidelines.

关键词： Mucoepidermoid Carcinoma sclerosing Tissue eosinop

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Diagnosis of IgG4-related sclerosing cholangitis

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World Journal of Gastroenterology 2013年第43期19卷 7661-7670页

作者： Takahiro Nakazawa Itaru Naitoh Kazuki Hayashi Katsuyuki Miyabe Shuya Simizu Takashi Joh Department of Gastroenterology and Metabolism Nagoya City University Graduate School of Medical SciencesNagoya 467-8601Japan

IgG4-related sclerosing cholangitis(IgG4-SC)is often associated with autoimmune pancreatitis.However,the diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis(PSC),and the presence of segmental stenosis suggests cholangiocarcinoma(CC).IgG4-SC responds well to steroid therapy,whereas PSC is only effectively treated with liver transplantation and CC requires surgical intervention.Since IgG4-SC was first described,it has become a third distinct clinical entity of sclerosing cholangitis.The aim of this review was to introduce the diagnostic methods for IgG4-SC.IgG4-SC should be carefully diagnosed based on a combination of characteristic clinical,serological,morphological,and histopathological features after cholangiographic classification and targeting of a disease for differential diagnosis.When intrapancreatic stenosis is detected,pancreatic cancer or CC should be ruled out.If multiple intrahepatic stenoses are evident,PSC should be distinguished on the basis of cholangiographic findings and liver biopsy with IgG4 immunostaining.Associated inflammatory bowel disease is suggestive of PSC.If stenosis is demonstrated in the hepatic hilar region,CC should be discriminated by ultrasonography,intraductal ultrasonography,bile duct biopsy,and a higher cutoff serum IgG4 level of 182 mg/dL.

关键词： IgG4-related sclerosing cholangitis Primary sclerosing cholangitis IgG4 sclerosing cholangitis

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