Description
Primary sclerosing cholangitis (PSC) is a chronic cholestatic (lack of bile flow) disease of the liver and bile ducts that is frequently progressive and can lead to end-stage liver disease. The disease is characterized by progressive inflammation, fibrosis, and stricturing of the intrahepatic (bile duct within the liver) and extrahepatic (outside of the liver) bile ducts.
Abnormal (with biliary stricture)
Normal
Epidemiology (prevalence in United States)
The prevalence of PSC in the general population has not been well-defined. The prevalence in the year 2000 was 20.9 cases per 100,000 men, and 6.3 cases per 100,000 women.
Inflammatory bowel disease (mostly ulcerative colitis) is present in about 73% of patients with PSC.
Gender
Approximately 70% of patients with PSC are men, with a mean age at diagnosis of 40 years. Women with PSC are generally diagnosed at an older age.
Genetics
There may be a genetic predisposition to PSC.
Pathogenesis (causes)
The cause of PSC is unknown.
Clinical manifestations (signs and symptoms)
The majority of patients with PSC have no symptoms at the time of diagnosis. The disease should be considered in patients with inflammatory bowel disease who have otherwise unexplained abnormal liver enzymes, particularly an elevation in serum alkaline phosphatase.
Fatigue and pruritus (general itching) are common features at presentation. In addition, fevers, chills, night sweats, and right upper quadrant pain occur in approximately 10 to 15% of patients when they are diagnosed. These features may represent episodic bacterial infections of the liver bile ducts from biliary obstruction rather than advanced disease. Liver biochemical tests may worsen during these episodes, but persistent jaundice (yellow eyes and/or skin) usually indicates more advanced disease.
Diagnosis
The diagnosis of PSC is made by visualizing multiple areas of stricturing (narrowing) throughout the biliary tree (liver bile ducts). These abnormalities are usually seen with a special x-ray and endoscopy called an ERCP. Abnormal liver bile ducts may also be suggested on ultrasound. A special type of MRI available at some hospitals called an MRCP may be an alternative to an endoscopic cholangiography ERCP.
Liver biopsy
A liver biopsy may support the diagnosis of PSC but is rarely used to make the diagnosis. The liver biopsy is helpful for staging the disease and determining prognosis. The staging system is listed below:
Liver biopsy
- Stage I: Enlargement, edema, and scarring of the portal triads with some piecemeal necrosis and damage to isolated bile ducts.
- Stage II: Expansion of portal triads with fibrosis extending into the surrounding parenchyma
- Stage III: Bridging fibrosis
- Stage IV: Cirrhosis
Prognosis
PSC is usually a progressive disorder that ultimately leads to the complications of cholestasis (lack of bile flow), cirrhosis, and liver failure. The average survival without liver transplantation after diagnosis is approximately 12 years. Survival is significantly worse for patients who have symptoms related to liver disease at the time they are diagnosed.
Treatment
The treatment of PSC is aimed at opening up dominant strictures (narrowed areas) with balloon dilation or stenting. Many patients have improvement in lab tests and symptoms, with initial successful stricture dilation in up to 85% of cases. However, the restenosis rate after successful dilation is 30 to 50 percent; as a result, patients require repeated treatments. Restenosis is first noted by episodes of right upper quadrant pain, fever, and/or jaundice (yellowing of the eyes and/or the skin).
The current treatment for PSC involves the combination of stricture dilation as needed and treatment with ursodeoxycholic acid (Ursodiol®, Actigall®).
Associated conditions
Cholestasis (lack of bile flow): The complications common to all of the chronic cholestatic liver diseases such as PSC include fatigue, pruritus (itching), steatorrhea (loose fatty stools), fat-soluble vitamin deficiencies (A, D, E, and K), and metabolic bone disease. Little is known about the pathogenesis of fatigue; nevertheless, it may become quite problematic and can be an indication for liver transplantation.
Pruritus (itching): Pruritus is a common symptom of PSC that can be extremely disabling and lead to severe excoriations and a decreased quality of life. The pathogenesis of pruritus in PSC, as in other disorders that cause cholestasis (lack of bile flow), is not clear.
Steatorrhea (loose fatty stools) and vitamin deficiency: Steatorrhea with concomitant fat-soluble vitamin deficiency in patients with PSC is generally thought to be due to decreased secretion of conjugated bile acids into the small intestine. However, associated conditions that may coexist with PSC, such as chronic pancreatitis and celiac disease, may also contribute to the genesis of steatorrhea.
Vitamin deficiency: Vitamin A deficiency has been reported in up to 82% of patients with advanced PSC, occasionally accompanied by night blindness. In addition, vitamin D and vitamin E deficiencies occur in approximately one-half of those with advanced disease. Thus, patients with PSC should be screened for fat-soluble vitamin deficiencies by determination of the prothrombin time and serum levels of vitamins A, D, and E. Supplemental therapy should be administered as necessary.
Metabolic bone disease: Metabolic bone disease, in particular osteoporosis, is a complication of advanced PSC. Calcium supplementation and measurement of vitamin D levels are generally recommended. For patients with more significant loss of bone density, medications may be used to correct the condition.
Cholangitis (infection of the liver bile ducts) and cholelithiasis (gallstones): Gallstones, due to cholesterol and/or pigment stones, may be present in up to one-third of patients with PSC. Gallstones in patients with PSC are treated the same way as in other patients. Attempts are made to remove gallstones only if they are causing obstruction of the major bile ducts; incidental gallstones in the gallbladder are not treated unless the clinical scenario dictates that they need to be removed.
Bacterial cholangitis (infection within the liver bile ducts) can occur in patients with PSC. The risk is greatest after endoscopic or surgical manipulation (including liver biopsy), but cholangitis can also develop spontaneously, particularly in patients with bile duct stones or obstructing strictures.
Cholangiocarcinoma (cancer of the bile ducts): Patients with PSC have a 10 to 15% lifetime risk of developing cholangiocarcinoma; those with inflammatory bowel disease and cirrhosis may be at highest risk.
Colon cancer: Patients with both PSC and ulcerative colitis have an increased risk of colon cancer and progression of neoplastic transformation. Therefore, yearly colonoscopies with biopsies are recommended.
Links for further information:
American Liver Foundation: www.liverfoundation.org
Cleveland Clinic Foundation: www.clevelandclinicmeded.com
Notes:
- Are all of your vaccines up to date?
- Do your bones need to be checked for thinning (osteoporosis)?
- Are you taking a daily multivitamin?
- Are you getting enough calcium and vitamin D?
- Do you need a colonoscopy or endoscopy?
© Copyright 1995-2008 The Cleveland Clinic Foundation. All rights reserved
Can't find the health information you’re looking for? Ask a Health Educator, Live!
Click here to go to the Department of Gastroenterology and Hepatology Web site.
Know someone who could use this information?....send them this link.
This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. This document was last reviewed on: 11/15/2005
