Related Experiment Video
Updated: May 14, 2026
04:38
Partial Bile Duct Ligation in the Mouse: A Controlled Model of Localized Obstructive Cholestasis
Published on: March 28, 2018
Primary sclerosing cholangitis: a review and update on therapeutic developments
James H Tabibian1, Keith D Lindor
1Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First St SW, Rochester, MN 55905, USA. tabibian.james@mayo.edu
Expert Review of Gastroenterology & Hepatology
|February 1, 2013
Summary
Primary sclerosing cholangitis (PSC) is a rare liver disease with no established treatments. This review examines past therapies and future directions for developing effective PSC treatments.
Area of Science:
- Hepatology
- Gastroenterology
- Immunology
Background:
- Primary sclerosing cholangitis (PSC) is a chronic, idiopathic liver disease causing bile duct inflammation and fibrosis.
- PSC can lead to cirrhosis, end-stage liver disease, and cholangiocarcinoma.
- Despite numerous clinical trials, no safe and effective pharmacotherapy for PSC has been established.
Purpose of the Study:
- To review previously tested pharmacologic agents for PSC.
- To discuss emerging fundamental concepts in PSC pathogenesis.
- To present viewpoints on the future evolution of PSC therapy development.
Main Methods:
- Comprehensive literature review of clinical trials and research on PSC pharmacotherapies.
- Analysis of pathogenic factors including genetic, immune, and microbial influences.
- Synthesis of current understanding and future perspectives on PSC treatment strategies.
Main Results:
- Decades of clinical trials involving nearly 20 pharmacotherapies have not yielded established treatments for PSC.
- PSC pathogenesis is complex and multifactorial, involving genetic, immune, and environmental factors, contributing to clinical heterogeneity.
- The complexity and heterogeneity of PSC are likely reasons for the negative results in past clinical trials.
Conclusions:
- Effective medical therapy for PSC remains critically needed but is not yet established.
- Novel insights into PSC pathogenesis and innovative approaches are necessary to improve treatment outcomes.
- Future therapeutic strategies for PSC will likely evolve based on a deeper understanding of its complex mechanisms and clinical heterogeneity.
Related Concept Videos
01:29Chronic Pancreatitis II: Collaborative Care
The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment:
Assessment:
01:17Chronic Bowel Disorders: Introduction
Chronic bowel diseases are a group of long-term conditions affecting the digestive tract, characterized by inflammation and damage to the gut lining. These conditions primarily include irritable bowel syndrome and inflammatory bowel disease.
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
Irritable Bowel Syndrome (IBS) is a common disorder affecting the gastrointestinal tract. The distinctive feature is recurrent abdominal pain associated with altered bowel movements, manifesting as constipation, diarrhea, or fluctuating between both. The...
01:24Cirrhosis II: Pathophysiology
Cirrhosis is a progressive chronic liver injury caused by prolonged inflammation, excessive fibrotic remodeling, and impaired regeneration. Over time, repeated hepatic insults disrupt the liver’s architecture and function, leading to reduced blood flow, impaired bile drainage, and diminished metabolic capacity.Pathophysiology of cirrhosisCirrhosis arises from three main responses to chronic liver damage: inflammation, immune activation, and hepatocyte death. These processes lead to structural...
01:20Cholecystitis
Cholecystitis is inflammation of the gallbladder, most commonly caused by obstruction of the cystic duct. This blockage prevents bile from draining, leading to gallbladder distension, inflammation, and potentially serious complications. This condition may present acutely or chronically and can happen with or without gallstones.EtiologyAbout 95% of cholecystitis cases are calculous, caused by gallstones blocking the cystic duct, leading to bile accumulation and inflammation of the gallbladder...
01:21Inflammatory Bowel Disease V: Surgical Management
Surgical interventions for inflammatory bowel disease (IBD), which includes ulcerative colitis and Crohn's disease, are essential in managing symptoms and addressing complications. The selection of surgical procedures is contingent upon the specific conditions and complications that stem from these illnesses.
Here are some common surgical interventions for IBD:
Here are some common surgical interventions for IBD:
01:25Chronic Pancreatitis I: Introduction
Chronic pancreatitis is a long-standing, relapsing inflammation of the pancreas, characterized by irreversible damage to the gland. It results in progressive destruction of the pancreatic parenchyma, fibrosis, and eventual loss of both exocrine and endocrine function. The disease may evolve gradually after multiple episodes of acute pancreatitis or develop independently.EtiologyChronic pancreatitis can arise from a variety of causes:Alcohol use is the leading cause, accounting for 70–80% of...