What is it?

This is a chronic progressive disorder that causes inflammation, fibrosis, and narrowing of the medium and large ducts, both inside and outside the liver that bring bile to the small intestine. These series of ducts is called the biliary tree.

The liver is an organ that is housed in the right upper side of your abdomen, mostly behind the ribs. It is about the size of a football and has many functions. It stores energy and nutrients, makes proteins, breaks down and filters substances, processes old red blood cells, and kills germs that enter the body through the intestines. The liver is also responsible for making bile. Bile is a fluid that is made up of water and other particles that is produced, stored in the gallbladder, and eventually arrives in the small intestine. Bile is important for the digestion and absorption of fats and some vitamins. Also, many waste products, such as bilirubin, a product that remains when old red blood cells are broken down, are excreted in bile and then eliminated from the body in a bowel movement.

The inflammation with primary sclerosing cholangitis (PSC) leads to narrowing of the bile ducts and can cause serious liver disease and increase the pressure in the blood vessels that flow through the liver, a condition called portal hypertension.

Who gets it?

The majority of affected people have Ulcerative Colitis (UC). But, only 5% of patients with UC have PSC. PSC is more common in men, 70% of affected people are male.

If you have a first-degree relative (parent, sibling, or child) with PSC, then you have an increased risk.

Why does it happen?

The cause is unknown, but it is likely that multiple things work together such as your genetics, your immune system, possible bacterial infection, or lack of blood flow.

What are the symptoms?

Most people are completely asymptomatic when they are diagnosed. Though symptoms can include:

  • Fatigue
  • Itchy skin
  • Fevers
  • Chills
  • Night sweats
  • Right upper abdominal pain

How is it diagnosed?

Blood work looking at liver functions tests may be elevated. Other blood tests looking at certain proteins in the blood may be obtained.

Cholangiography. This is when dye is sent into the ducts and pictures are taken showing the outlines. It can be performed with a magnetic resonance imaging (MRI) or with an endoscopic retrograde cholangiopancreatography (ERCP); a procedure where a camera is placed in your mouth down your throat to the part of the small intestine where the ducts from the pancreas and liver join. The dye can be injected directly. With PSC, there are areas of narrowing and expansion of the bile ducts inside and/or outside of the liver.

Ultrasound may be used, but usually better pictures are needed. A tissue sample of the liver, a biopsy, may be obtained.

How is it treated?

The two big goals of treatment are to slow and reverse the disease and manage any complications.

There is no current treatment that slows the disease. Many drugs that decrease the immune system’s activity and inflammation have been studied, but none have been proven effective across the board. Because it is a progressive disorder, ultimately there are complications because the flow of bile from the liver to the small intestine being blocked (cholestasis) can lead to failure of the liver. A liver transplant is the best treatment for advanced disease.

What are the complications?

Problems linked to the flow of bile being blocked from the liver to the small intestine

It is not clear why this happens, but it can be very severe. It may be due to the buildup of bile acids. Fatty diarrhea and vitamin deficiency. This may be due to decreased amounts of bile acids in the small intestine. Because fat is not being absorbed as well, there may be difficulties absorbing vitamins that are dissolved in fat such as vitamin A, D, E, and K.

Bone disease
Narrowing of the ducts. With significant narrowing, often symptoms are like a blockage with yellowing of the skin, itchiness, malabsorption, and potentially an infection called ascending cholangitis. Medicines are not effective to treat narrowing, but dilation (stretching) or placing a stent may be tried. The narrowing can also be due to cancer, so testing will be done to rule out a cancer.

Stones due to cholesterol or pigment are present in up to one-third of patients and can cause blockages. Ultrasound can be used to see if there is a blockage, but then usually an ERCP will be done to see if that blockage is due to just a narrowing, a stone, or a cancer. If stones are present, this is treated like any other patient.

Infection of the ducts.
This can happen at random, but the risk is greatest after surgery or an ERCP.

There is an increased risk for cholangiocarcinoma, gallbladder cancer, and if cirrhosis is also present, hepatocellular carcinoma.

The risk of colon cancer is also increased in patients who also have UC.

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