Related Experiment Videos
Primary sclerosing cholangitis.
Joy Worthington1, Roger Chapman
1Department of Gastroenterology, John Radcliffe Hospital, Headington, Oxford, OX3 9DU, UK. joyworthington@hotmail.com
Orphanet Journal of Rare Diseases
|October 26, 2006
Summary
Primary sclerosing cholangitis (PSC) is a rare liver disease causing bile duct inflammation and scarring. Early diagnosis in asymptomatic patients offers a better survival rate, with liver transplantation as the main treatment for advanced stages.
Area of Science:
- Hepatology
- Gastroenterology
- Immunology
Background:
- Primary sclerosing cholangitis (PSC) is a chronic, immune-mediated liver disease characterized by biliary tree inflammation and fibrosis.
- It affects men twice as often as women, with a mean age at diagnosis of 40 years.
- PSC is associated with inflammatory bowel disease, particularly ulcerative colitis.
Purpose of the Study:
- To summarize the key aspects of Primary Sclerosing Cholangitis (PSC).
- To highlight the diagnostic criteria and prognosis of PSC.
- To discuss current therapeutic options for PSC.
Main Methods:
- Diagnosis relies on characteristic cholangiographic and histological findings.
- Exclusion of secondary causes of sclerosing cholangitis is essential.
- Epidemiological data on incidence and prevalence are reported.
Main Results:
- The disease often presents insidiously with symptoms like fatigue or pruritus, or may be asymptomatic.
- Progression to cirrhosis, liver failure, and cholangiocarcinoma (8-30% of patients) is common.
- Median survival is 12 years for symptomatic patients, but over 70% survival at 16 years for asymptomatic patients.
Conclusions:
- Liver transplantation is the only definitive treatment for end-stage PSC.
- High-dose ursodeoxycholic acid may offer some benefit.
- Understanding PSC's natural history and risk factors is crucial for patient management.
Related Concept Videos
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...
01:21Chronic Pancreatitis II: Pathophysiology
Chronic pancreatitis is a progressive and irreversible inflammation of the pancreas, most often caused by long-term alcohol abuse, but it can also be related to ductal obstruction, smoking, or genetic factors.Chronic pancreatitis occurs when the pancreas is repeatedly exposed to harmful agents like alcohol, smoking, ductal obstruction, or genetic predisposition. These factors lead to the release of toxic metabolites and inflammatory cytokines, sustaining chronic inflammation in the pancreatic...
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...
01:25Chronic 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...
01:24Chronic Pancreatitis I: Introduction
The pancreas, an elongated and flat gland situated behind the stomach, serves a vital function in digesting food and managing blood sugar levels.
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
Acute pancreatitis arises suddenly and lasts for a brief duration, while chronic pancreatitis is a long-term affliction...
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: