Primary Sclerosing Cholangitus (PSC)


Fact: Many people may have the disease for years before symptoms develop

What is primary sclerosing cholangitis?
Primary sclerosing cholangitis (PSC) is a chronic, or long-term, disease that slowly damages the bile ducts. Bile is a digestive liquid that is made in the liver. It travels through the bile ducts to the gallbladder and the small intestine, where it helps digest fats and fatty vitamins.

In patients with PSC, the bile ducts become blocked due to inflammation and scarring or fibrosis. This causes bile to accumulate in the liver, where it gradually damages liver cells and causes cirrhosis, or fibrosis of the liver. As cirrhosis progresses and the amount of scar tissue in the liver increases, the liver slowly loses its ability to function. The scar tissue may block drainage of the bile ducts leading to infection of the bile.

PSC advances very slowly. Many patients may have the disease for years before symptoms develop. Symptoms may remain at a stable level, they may come and go, or they may progress gradually. Liver failure may occur 10-15 years after diagnosis, but this may take even longer for some PSC patients. Many people with PSC will ultimately need a liver transplant, typically about 10 years after being diagnosed with the disease. PSC may also lead to bile duct cancer. Endoscopy and MRI tests may be done to monitor the disease.

Symptoms
Primary sclerosing cholangitis is often diagnosed before symptoms appear when a routine blood test or an X-ray taken for an unrelated condition shows liver abnormalities.
Early symptoms often include:

  • Fatigue
  • Itching

Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years, but there's no reliable way to predict how quickly or slowly the disease will progress for any individual. Signs and symptoms that may appear as the disease progresses include:

  • Pain in the upper right part of the abdomen
  • Fever
  • Chills
  • Night sweats
  • Enlarged liver
  • Weight loss
  • Yellow eyes and skin (jaundice)

Causes
It's not clear what causes primary sclerosing cholangitis. An immune system reaction to an infection or toxin may trigger the disease in people who are genetically predisposed to it.

A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term that includes ulcerative colitis and Crohn's disease. Primary sclerosing cholangitis and inflammatory bowel disease don't always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. Somewhat less often, people being treated for inflammatory bowel disease turn out to have primary sclerosing cholangitis as well. And rarely, people with primary sclerosing cholangitis develop inflammatory bowel disease only after having a liver transplant.

Risk factors
Factors that may increase the risk of primary sclerosing cholangitis include:

  • Your age. Primary sclerosing cholangitis can occur at any age, but it's most often diagnosed between the ages of 30 and 50.
  • Your sex. Primary sclerosing cholangitis occurs more often in men.
  • Inflammatory bowel disease. A large proportion of people with primary sclerosing cholangitis also have inflammatory bowel disease.
  • Your geographical location. People in the United States and Northern Europe have a higher risk of primary sclerosing cholangitis than do people in Asia and Southern Europe.


Complications
Complications of primary sclerosing cholangitis may include:

  • Liver disease and failure. Chronic inflammation of the bile ducts throughout your liver can lead to tissue scarring (cirrhosis), liver cell death and, eventually, loss of liver function.
  • Repeated infections. If scarring of the bile ducts slows or stops the flow of bile out of the liver, you may experience frequent infections in the bile ducts. The risk of infection is particularly high after you've had a surgical procedure to expand a badly scarred bile duct or remove a stone blocking a bile duct.
  • Portal hypertension. Your portal vein is the major route for blood flowing from your digestive system into your liver. Portal hypertension refers to high blood pressure in this vein. Portal hypertension can cause fluid from the liver to leak into your abdominal cavity (ascites). It can also divert blood from the portal vein to other veins, causing these veins to become swollen (varices). Varices are weak veins and tend to bleed easily, which can be life-threatening.
  • Thinning bones. People with primary sclerosing cholangitis may experience thinning bones (osteoporosis). Your doctor may recommend a bone density exam to test for osteoporosis every few years. Calcium and vitamin D supplements may be prescribed to help prevent bone loss.
  • Bile duct cancer. If you have primary sclerosing cholangitis, you have an increased risk of developing cancer in the bile ducts or gallbladder.
  • Colon cancer. People with primary sclerosing cholangitis associated with inflammatory bowel disease have an increased risk of colon cancer. If you've been diagnosed with primary sclerosing cholangitis, your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.

Tests and diagnosis

Tests and procedures used to diagnose primary sclerosing cholangitis include:

  • Liver function blood tests. A blood test to check your liver function, including levels of your liver enzymes, can give your doctor clues about your diagnosis.
  • MRI of your bile ducts. Magnetic resonance cholangiopancreatography (koh-lan-jee-o-pan-cree-uh-TOG-ruh-fee) uses magnetic resonance imaging (MRI) to make images of your liver and bile ducts.
  • X-rays of your bile ducts. You may need a type of bile duct X-ray called endoscopic retrograde cholangiopancreatography (ERCP) in addition to or instead of an MRI. To make your bile ducts visible on an X-ray, your doctor uses a flexible tube passed down your throat to inject dye into the area of your small intestine where your bile ducts empty. ERCP is the test of choice if signs and symptoms persist despite no abnormalities on MRI. ERCP is often the initial test if you're unable to have an MRI because of a metal implant in your body.
  • Testing a sample of liver tissue. A liver biopsy is a procedure to remove a piece of liver tissue for laboratory testing. Your doctor inserts a needle through your skin and into your liver to extract a tissue sample. Liver biopsy can help determine the extent of damage to your liver. The test is used only when the diagnosis of primary sclerosing cholangitis is still uncertain after less invasive tests.


How is PSC treated?
There is no cure or specific treatment for PSC. The itching associated with the disease can be relieved with medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.

​Lifestyle and home remedies
If you've been diagnosed with primary sclerosing cholangitis, take steps to care for your liver, such as:

  • Don't drink alcohol.
  • Get vaccinated against hepatitis A and B.
  • Use care with chemicals at home and at work.
  • Maintain a healthy weight.
  • Follow directions on all medications, both prescription and over the counter. Make sure your pharmacist and any doctor prescribing for you know that you have a liver disease.
  • Talk to your doctor about any herbs or supplements you're taking since some can be harmful to your liver.

Information on this page taken directly from:
American Liver Foundation
Mayo Clinic

Want to learn more?
The following are organizations and/or websites dedicated to providing information and education surrounding Primary Sclerosing Cholangitus (PSC)
. These organizations are dedicated to research, education, awareness, and/or support. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.


American Liver Foundation
British Liver Trust
Mayo Clinic
Medicinenet.com
NIDDK
WebMD