Primary sclerosing cholangitis is a progressive disease that damages your bile ducts and your liver. It causes chronic inflammation and scarring. Scar tissue eventually restricts the flow of bile through the ducts and stops the liver from functioning properly.
Primary sclerosing cholangitis (PSC) is a disease of the bile ducts. It causes chronic inflammation in your bile ducts (cholangitis), which eventually causes scarring (sclerosis). Scar tissue in your bile ducts causes them to narrow, which restricts the flow of bile through the ducts (biliary stricture). When bile can’t flow, it backs up into your liver, where it came from. This does progressive damage to your liver.
Yes. While you might not have symptoms at first, PSC is a progressive disease that gets worse over time. When bile stalls in your bile ducts (cholestasis), it can begin to leak bile toxins into your bloodstream, which makes you sick. It also does progressive damage to your liver. Within 10 to 15 years, this can lead to liver failure. You can’t live without a liver. While there are temporizing treatments, there is currently no cure for PSC, except a liver transplant.
“Primary” means that this disease is the original cause of inflammation and scarring of your bile ducts. There’s no other cause but the disease. In secondary sclerosing cholangitis, inflammation (cholangitis) and scarring (sclerosis) of your bile ducts are secondary effects caused by something else. Some causes of secondary sclerosing cholangitis include:
Primary biliary cholangitis (PBC) is the current name for what was previously called primary biliary cirrhosis.
PBC and PSC are both progressive bile duct-related liver diseases, with similar symptoms and effects. Both diseases cause the gradual deterioration of your bile ducts. Destruction and scarring in your bile ducts cause them to narrow and the flow of bile to stall. The bile begins to back up into your liver, where it causes further damage. This leads to scarring and eventually, cirrhosis of the liver.
Here are the primary differences:
PSC is rare. It’s estimated to occur in about 1 in 10,000 people worldwide.
PSC occurs in people assigned male at birth twice as often as in people assigned female at birth. It’s most often diagnosed around the age of 40. About 80% of people with PSC also have inflammatory bowel disease, most often ulcerative colitis. It’s more likely to occur in people with a family history of the disease.
It’s not entirely clear, but it appears to involve a combination of factors, including:
Doctors believe that PSC may be a type of autoimmune disease. That means it causes your body’s immune system to attack healthy cells as invaders. Inflammation is one of the tools of your immune system. It’s supposed to be an acute response to an attack, but when inflammation becomes chronic, it tends to be a sign of disease.
Doctors have also noticed that people who have PSC are prone to other autoimmune diseases, including:
It appears that certain environmental factors (such as toxic exposure) may trigger an autoimmune response in people with certain genetic profiles.
The signs and symptoms of PSC change as the disease becomes more advanced. Up to 50% of people may have no symptoms at all at the time of diagnosis. PSC is often found by accident when testing for other conditions. The first symptoms to develop tend to be vague. They may include:
Later-stage symptoms may include:
Primary sclerosing cholangitis is a slow progressing disease. But as your bile ducts and liver become increasingly scarred, their functions will increasingly fail. This can lead to:
When your bile ducts become significantly blocked, they won’t be able to deliver bile to your small intestine to help with digestion as they normally would. Bile is necessary to help break down fats in your intestines and to absorb fat-soluble vitamins (A, D, E and K). Without it, your digestive system may have trouble processing fats, and you may not be able to absorb these essential vitamins from your food. This can lead to a variety of additional complications, including:
Progressive cirrhosis of the liver reduces blood flow through your liver, which increases pressure in the portal vein that runs through your digestive system. This pressure causes other veins in your esophagus and abdomen to enlarge and makes them vulnerable to bursting, which can cause internal bleeding.
Advanced PSC leads to an increased risk of developing several cancers, including:
It’s often found by accident while testing for something else. Up to 50% of people may not have any symptoms at the time of diagnosis. Early signs of the disease may show up on a blood test or imaging test. Images of bile ducts with PSC show certain distinctive features. A blood test may show high levels of alkaline phosphatase or certain antibodies that indicate an immune response in your bile ducts. White blood cell elevation is usually a sign of infection in the liver.
To confirm the disease, your healthcare provider may suggest a more specific test, such as a:
Currently, there is no treatment that can slow or stop primary sclerosing cholangitis from progressing. You can treat some of the symptoms and complications directly. For example, your healthcare provider may prescribe:
Your healthcare provider will also keep your liver and bile ducts under surveillance. As the disease progresses, they may be able to periodically intervene by opening up a blocked bile duct. They can do this through an ERCP (endoscopic retrograde cholangiopancreatography), a type of exam that allows access to your bile ducts without surgery.
An ERCP is a type of endoscopy, which means it examines your organs using an endoscope, a tiny lighted camera attached to a long, flexible tube. Under anesthesia, your healthcare provider advances a tube down your throat into your abdominal cavity. They can pass tiny instruments through the tube to treat your bile ducts. Your technician can dilate your bile duct with a balloon, or prop it open with a stent. If the blockage is not accessible by ERCP then they obtain access directly through the skin, a procedure called percutaneous trans-hepatic cholangiography (PTHC).
This is a temporary solution, however. Over the course of 10 to 20 years, primary sclerosing cholangitis eventually progresses from early to late-stage liver disease, and finally, liver failure. Your healthcare provider will monitor your liver damage to determine when you should begin to consider liver transplantation. You’ll need to pass certain qualifications to get on a liver transplantation waiting list.
After diagnosis, the average life expectancy ranges between 10 and 20 years. A liver transplant can give you a new lease on life. However, in 15% to 20% of cases, PSC may return after a liver transplant. When this happens, the new liver may fail. The average life expectancy in this case is about nine months.
Another factor that can affect life expectancy is cancer. If cancer develops as a complication of PSC, you may not be a good candidate for a liver transplant. In carefully selected cases, healthcare providers may attempt to treat cancer first with radiation or chemotherapy and then attempt a liver transplant.
Lifestyle choices can help to manage the fatigue that’s common with PSC and prevent additional damage to your liver. For example:
A note from Cleveland Clinic
Primary sclerosing cholangitis is rare, unpredictable and unpreventable. It progresses slowly and often without early symptoms. But if you visit your healthcare provider periodically for routine screenings, you may discover it well before it begins to affect your life. This is your chance to implement lifestyle choices that may help to preserve the health of your liver longer. You’ll also begin the process of long-term medical maintenance, including regular testing and interventions when necessary.
Last reviewed by a Cleveland Clinic medical professional on 07/17/2022.
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