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.
What causes primary sclerosing cholangitis?
It’s not entirely clear, but it appears to involve a combination of factors, including:
Genetics.
Environment.
Immune cells.
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.
What are the signs and symptoms of primary sclerosing cholangitis?
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:
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:
Metabolic diseases
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.
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:
Medications to treat itchy skin (pruritus).
Supplements to treat vitamin deficiencies.
Antibiotics to treat infections.
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.
How is primary sclerosing cholangitis diagnosed?
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:
Liver function test. These blood tests look for high levels of particular liver enzymes. High levels of alkaline phosphatase may indicate PSC.
Magnetic resonance cholangiopancreatography (MRCP). This test uses magnetic resonance imaging (MRI) to produce detailed pictures of the biliary tree (your liver, gallbladder and bile ducts). This is the first-line imaging test for PSC because it’s non-invasive and avoids radiation exposure. Occasionally, it might not reveal an early or mild case of the disease, and you might need another kind of imaging test.
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