Bile Acids and Cholestatic Liver Disease 2: Primary Sclerosing Cholangitis

2017 ◽  
pp. 121-134
Author(s):  
Takahiro Nakazawa
Keyword(s):  
Liver Disease ◽  
Bile Acids ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease

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.

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

scholarly journals Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

2020 ◽  
Vol 25 (2) ◽  
pp. 107-117
Author(s):  
Dong-Won Ahn
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Acute Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases, in which liver transplantation is required ultimately in most patients. Imaging studies such as magnetic resonance cholangiopancreatography have important role in diagnosis in most cases of PSC. PSC is usually accompanied by inflammatory bowel disease and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in endstage biliary cirrhosis requiring liver transplantation. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC and risk stratification is important to ensure all patients receive a personalised approach to their care. Medical therapy using ursodeoxycholic acid (UDCA) or obeticholic acid (OCA) has an important role to reduce the progression to end-stage liver disease in PBC.

scholarly journals Medical Management of Chronic Cholestatic Liver Diseases

2000 ◽  
Vol 14 (suppl d) ◽  
pp. 93D-98D ◽  
Author(s):  
Andrea A Gossard ◽  
Keith D Lindor
Keyword(s):  
Liver Disease ◽  
Primary Biliary Cirrhosis ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Biliary Obstruction ◽  
Sclerosing Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis ◽  
Management Options ◽  
Extrahepatic Biliary Obstruction

The purpose of the present review is to discuss the diagnosis and management of cholestatic liver diseases. Differential diagnoses to consider are described, including causes of extrahepatic biliary obstruction such as gallstones, strictures, extrabiliary malignancies and pancreatitis. In addition, diseases that cause intrahepatic cholestasis such as primary biliary cirrhosis, primary sclerosing cholangitis, hepatocellular diseases and a variety of miscellaneous causes including drugs that may cause cholestasis are discussed. Primary biliary cirrhosis and primary sclerosing cholangitis are reviewed in detail, and management options are identified. The prognosis of patients with these diseases is discussed, and the Mayo Mathematical Models in Cholestatic Liver Disease for both primary biliary cirrhosis and primary sclerosing cholangitis are provided. Finally, management options for the complications of cholestasis are provided.

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 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 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.

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