scholarly journals Management of primary sclerosing cholangitis and its complications: an algorithmic approach

2020 ◽  
Author(s):  
Michal Prokopič ◽  
Ulrich Beuers
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Treatment Options ◽  
Diagnostic Procedures ◽  
Disease Recurrence ◽  
Cholestatic Liver Disease ◽  
Diagnostic Challenge ◽  
Algorithmic Approach ◽  
End Stage

AbstractPrimary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.

scholarly journals Primary sclerosing cholangitis

2008 ◽  
Vol 22 (8) ◽  
pp. 689-698 ◽  
Author(s):  
Marina G Silveira ◽  
Keith D Lindor
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Serum Alkaline Phosphatase ◽  
Cholestatic Liver Disease ◽  
Biliary Strictures ◽  
Vitamin Deficiencies ◽  
Metabolic Bone ◽  
High Doses ◽  
End Stage

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of the bile ducts, resulting in end-stage liver disease and reduced life expectancy. PSC primarily affects young and middle-aged men, often in association with underlying inflammatory bowel disease. The etiology of PSC includes immune-mediated components and elements of undefined nature. A cholestatic picture of liver biochemistries with elevations in serum alkaline phosphatase, nonspecific autoantibodies such as perinuclear antineutrophilic antibody, antinuclear antibodies and smooth muscle antibodies, and diffuse multifocal biliary strictures, resulting in a ‘beaded’ appearance on radiographic studies, are the hallmarks of the disease. No effective medical therapy is currently available, although clinical studies are in progress. Ursodeoxycholic acid at high doses (28 mg/kg/day to 30 mg/kg/day) is the most promising agent but is unproven so far. Liver transplantation is currently the only life-extending therapy for patients with end-stage disease, although recurrent disease can be observed in the transplanted liver. The multiple complications of PSC include pruritus, fatigue, vitamin deficiencies, metabolic bone disease, peristomal varices, bacterial cholangitis, dominant biliary strictures, gallbladder stones and polyps, and malignancy, particularly cholangiocarcinoma, which is the most lethal complication of PSC.

Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

2017 ◽  
Vol 37 (04) ◽  
pp. 305-313 ◽  
Author(s):  
Cynthia Levy ◽  
Eric Martin
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Graft Survival ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Immune Mediated ◽  
End Stage ◽  
Patient And Graft Survival

AbstractPrimary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

scholarly journals The PSC scientific community resource: an asset for multi-omics interrogation of primary sclerosing cholangitis

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Ahmad Hassan Ali ◽  
Brian D. Juran ◽  
Erik M. Schlicht ◽  
Jackie K. Bianchi ◽  
Bryan M. McCauley ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Mayo Clinic ◽  
Sclerosing Cholangitis ◽  
Scientific Community ◽  
Treatment Options ◽  
Biological Response ◽  
Clinical Information ◽  
Cholestatic Liver Disease ◽  
Community Resource

Abstract Background Primary sclerosing cholangitis (PSC) is a rare, chronic cholestatic liver disease that often progresses to end-stage liver disease and/or the development of hepatobiliary neoplasia. Lack of prognostic tools and treatment options for PSC is driven in part by our poor understanding of its pathogenesis, which is thought to be complex, the interaction of genetic variants, environmental influences and biological response throughout the course of disease. The PSC Scientific Community Resource (PSC-SCR) seeks to overcome previous shortcomings by facilitating novel research in PSC with the ultimate goals of individualizing patient care and improving patient outcomes. Methods PSC patients who receive their health care at Mayo Clinic or a collaborating site are identified by chart review and invited in person or by mail to participate. Non-Mayo patients are offered enrollment if they provide sufficient access to their medical records to evaluate inclusion/exclusion criteria. Controls without liver disease are identified with assistance of the Mayo Clinic Biobank. Participant consent is obtained at the beginning of the recruitment process by mail-in, electronic or face-to-face protocols. Clinical data is extracted from the medical record by qualified physicians and entered in a custom designed database. Participants fill out a custom-designed, comprehensive questionnaire, which collects scientifically relevant demographic and clinical information. Biospecimens are collected using mail-in kits thar are returned via overnight carrier service and processed by the biospecimen accessioning and processing facility at Mayo Clinic, which coordinates sample transfers and provides required sample preparation services. The resource is currently being utilized to perform omics-scale projects investigating the exposome, metabolome, methylome, immunome and microbiome in PSC. Datasets and residual biospecimens will be shared with researchers proposing scientifically sound PSC-focused research with approval of the appropriate review boards. Discussion Patient-based studies leveraging the latest technologies for targeted and wide-scale interrogation of multiple omics layers offer promise to accelerate PSC research through discovery of unappreciated aspects of disease pathogenesis. However, the rarity of PSC severely limits such studies. Here we describe our effort to overcome this limitation, the PSC-SCR, a repository of patient biospecimens coupled with clinical and omics data for use by the broader PSC research community.

scholarly journals PSC Scientific Community Resource The PSC Scientific Community Resource: An Asset for Multi-Omics Interrogation of Primary Sclerosing Cholangitis

2021 ◽  
Author(s):  
Ahmad Hassan Ali ◽  
Brian D. Juran ◽  
Erik M. Schlicht ◽  
Jackie K. Bianchi ◽  
Bryan M. McCauley ◽  
...  
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Mayo Clinic ◽  
Sclerosing Cholangitis ◽  
Scientific Community ◽  
Treatment Options ◽  
Biological Response ◽  
Clinical Information ◽  
Cholestatic Liver Disease ◽  
Community Resource

Abstract Background: Primary sclerosing cholangitis (PSC) is a rare, chronic cholestatic liver disease that often progresses to end-stage liver disease and/or the development of hepatobiliary neoplasia. Lack of prognostic tools and treatment options for PSC is driven in part by our poor understanding of its pathogenesis, which is thought to be complex, the interaction of genetic variants, environmental influences and biological response throughout the course of disease. The PSC Scientific Community Resource (PSC-SCR) seeks to overcome previous shortcomings by facilitating novel research in PSC with the ultimate goals of individualizing patient care and improving patient outcomes.Methods: PSC patients who receive their health care at Mayo Clinic or a collaborating site are identified by chart review and invited in person or by mail to participate. Non-Mayo patients are offered enrollment if they provide sufficient access to their medical records to evaluate inclusion/exclusion criteria. Controls without liver disease are identified with assistance of the Mayo Clinic Biobank. Participant consent is obtained at the beginning of the recruitment process by mail-in, electronic or face-to-face protocols. Clinical data is extracted from the medical record by qualified physicians and entered in a custom designed database. Participants fill out a custom-designed, comprehensive questionnaire, which collects scientifically relevant demographic and clinical information. Biospecimens are collected using mail-in kitsthar are returned via overnight carrier service and processed by the biospecimen accessioning and processing facility at Mayo Clinic, which coordinates sample transfers and provides required sample preparation services.The resource is currently being utilized to perform omics-scale projects investigating the exposome, metabolome, methylome, immunome and microbiome in PSC. Datasets and residual biospecimens will be shared with researchers proposing scientifically sound PSC-focused research with approval of the appropriate review boards.Discussion:Patient-based studies leveraging the latest technologies for targeted and wide-scale interrogation of multiple omics layers offer promise to accelerate PSC research through discovery of unappreciated aspects of disease pathogenesis. However, the rarity of PSC severely limits such studies. Here we describe our effort to overcome this limitation, the PSC-SCR, a repository of patient biospecimens coupled with clinical and omics data for use by the broader PSC research community.

scholarly journals Recent advances in understanding bile duct remodeling and fibrosis

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1165
Author(s):  
Marinda Scrushy ◽  
April O'Brien ◽  
Shannon Glaser
Keyword(s):  
Liver Disease ◽  
Bile Duct ◽  
Liver Transplant ◽  
Biliary Atresia ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Treatment Options ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Age Range

Cholestatic liver disease encompasses a detrimental group of diseases that are non-discriminatory in nature. These diseases occur over every age range from infancy (biliary atresia) to geriatrics (hepatitis). They also cover both genders in the form of primary sclerosing cholangitis in men and primary biliary cholangitis in women. Oftentimes, owing to the disease progression and extensive scarring, the treatment of last resort becomes a liver transplant. In this review, we will briefly discuss and explore new avenues of understanding in the progression of cholestatic liver disease and possible therapeutic targets for intervention. The greater our understanding into the idiopathic nature of cholestatic liver disease, the better our chances of discovering treatment options to halt or reverse the progression, reducing or eliminating the need for expensive and risky transplants.

Cholestatic Liver Injury: Care of Patients With Primary Biliary Cholangitis or Primary Sclerosing Cholangitis

2016 ◽  
Vol 27 (4) ◽  
pp. 441-452 ◽  
Author(s):  
Laurie Larson ◽  
Michelle James ◽  
Andrea Gossard
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Disease Recurrence ◽  
Outpatient Setting ◽  
Primary Biliary Cholangitis ◽  
Malignant Neoplasms ◽  
Cholestatic Liver Disease ◽  
Gallbladder Polyps ◽  
Increased Risk ◽  
Common Causes

The most common causes of chronic cholestatic liver disease are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both disease processes are characterized by a destruction of intrahepatic and/or extrahepatic biliary ducts. The etiology is not entirely clear; however, there is an underlying autoimmune component contributing to both disease processes. Although PBC and PSC are often diagnosed and managed in the outpatient setting, in some instances, a patient may have jaundice, fatigue, and pruritus requiring evaluation and determination of the cholestatic cause. Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms. Liver transplant has the potential for improving quality of life, although disease recurrence is a risk.

scholarly journals Role of the Microbiota and Antibiotics in Primary Sclerosing Cholangitis

2013 ◽  
Vol 2013 ◽  
pp. 1-7 ◽  
Author(s):  
James H. Tabibian ◽  
Jayant A. Talwalkar ◽  
Keith D. Lindor
Keyword(s):  
Inflammatory Bowel Disease ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Clinical Evidence ◽  
Cholestatic Liver Disease ◽  
Concise Review ◽  
Inflammatory Bowel ◽  
Considerable Morbidity

Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, cholestatic liver disease with considerable morbidity and mortality and no established pharmacotherapy. In addition to the long-recognized association between PSC and inflammatory bowel disease, several lines of preclinical and clinical evidence implicate the microbiota in the etiopathogenesis of PSC. Here we provide a concise review of these data which, taken together, support further investigation of the role of the microbiota and antibiotics in PSC as potential avenues toward elucidating safe and effective pharmacotherapy for patients afflicted by this illness.

scholarly journals Problem Diagnostik dan Tata Laksana Primary Sclerosing Cholangitis

2017 ◽  
Vol 3 (3) ◽  
pp. 158
Author(s):  
Rezky Aulia Nurleili ◽  
Intan Airlina ◽  
Anna Mira Lubis
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Best Management ◽  
High Incidence ◽  
Male Patients

Primary sclerosing cholangitis (PSC) merupakan penyakit hati kolestasis kronik dengan insiden yang cukup tinggi di Amerika yaitu sebesar 1/100.000 penduduk namun angka kejadiannya di Indonesia belum pernah dipublikasikan. Belum didapatkan tatalaksana yang terbaik pada penyakit ini. Pada artikel ini akan dibahas mengenai kasus PSC pada pasien laki-laki berusia 49 tahun di Rumah Sakit dr. Cipto Mangunkusumo (RSCM) Jakarta dan tatalaksana yang dilakukan.Kata Kunci: PSC, tata laksana Diagnostic and Treatment Problems of Primary Sclerosing CholangitisPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with high incidence reported in America (1/100.000 population), but there is still no data available in Indonesia. The best management of this disease is still not found. Thus, in this article author will discuss about the case of PSC in 49-years male patients hospitalized in Cipto Mangunkusumo hospital Jakarta and its treatment. Keywords: PSC, treatment

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