Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC)  is diagnosed and treated by the GI Division of Premier Medical Group. Primary sclerosing cholangitis (PSC) is a disease that damages and blocks bile ducts inside and outside the liver. Bile is the liquid made in your liver and the bile ducts are the tubes that carry bile out of the liver. From there the bile travels to the gallbladder and small intestine, helping to break down fat in food in the intestine. PSC is a chronic disease that can last for years, even decades, and worsens over time.

Your liver produces bile, among many other functions. Bile is a watery liquid which is important for digesting food in the intestine, particularly fat, and ridding the body of toxins. Primary sclerosing cholangitis occurs when there is inflammation (cholangitis) in the bile ducts. This can lead to scarring (sclerosis) and narrowing of the ducts. The result is a build-up of bile in the liver because it cannot be released to the gallbladder and small intestine. Primary sclerosing can eventually damage liver cells and the scar tissue can spread throughout the liver, causing cirrhosis and liver failure.

Roughly 70 percent of people with primary sclerosing cholangitis have inflammatory bowel disease (IBD), most commonly a form of the condition known as ulcerative colitis. People with primary sclerosing are also more prone to have an autoimmune disorder, such as celiac disease, thyroid disease, or type 1diabetes; they also have a higher risk of developing cancer, especially in the bile ducts.

What causes primary sclerosing cholangitis?

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The causes of primary sclerosing cholangitis are not known. It affects more men than women; about 70 percent of PSC patients are men. Genes, immune system, bacteria, and viruses may play a role. PCS is not a common disease, but recent studies are showing that we are seeing more of it. Three out of four people with PSC also have an IBD disease such as ulcerative colitis, although the link between the two diseases has not been explained.

In PSC, the bile ducts get inflamed scarred and thickened (sclerotic) narrowed and eventually obstructed, not allowing for the normal passage of the bile into the liver and intestines where it mixes with food.

What are the symptoms of primary sclerosing cholangitis?

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Most patients with early primary sclerosing cholangitis have no symptoms. Because PSC progresses slowly, a person can have the disease for years before any symptoms develop. Typically, the disease is only recognized when elevated blood level of liver enzymes shows up during routine blood work.  When PSC progresses, patients usually develop the following symptoms:

  • Abdominal pain
  • Itching (pruritus)
  • Fatigue
  • Yellowing of the skin or whites of the eyes
  • Chills and fever

People who have the autoimmune form of primary sclerosing cholangitis tend to get symptoms much more rapidly than the majority of patients with the more idle form of primary sclerosing cholangitis. As the disease progresses, it causes cirrhosis of the liver and liver failure; leading to the consideration of liver transplantation. With advanced cirrhosis, patients may develop frequent infections, fluid in the ankles and the abdomen (ascites). Some patients also suffer from mental confusion, all signs of liver failure.

How is primary sclerosing cholangitis diagnosed?

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The first step to diagnose PSC is a blood test to check levels of liver enzymes. Other testing may include ultrasound exams and a liver biopsy. Your doctor may order another diagnostic exam (Cholangiography) to confirm the PSC. This can be achieved with the following procedures:

  • Endoscopic retrograde cholangiopancreatography (ERCP): An endoscopic procedure using dye injected into the bile ducts to determine if they are blocked.
  • Percutaneous transhepatic cholangiography: Dye is injected through a tube into the duct and x-rays are taken.
  • Magnetic resonance cholangiopancreatography (MRCP): This uses MRI imaging to obtain pictures of the bile ducts. MRCP does not involve using x rays or inserting instruments into the body. This safe and painless test is increasingly used for diagnosis.

How is primary sclerosing cholangitis treated?

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At this time, there is no cure or specific treatment for PSC. Many treatments have been studied, but none have shown to cure or slow the progress of PSC. Treatments seem to relieve the symptoms and manage any complications, but do not cure the disease. If the liver begins to fail, liver transplant may be an option. Many patients with PSC need to take vitamin supplements.  In some cases bile duct surgery may be necessary to improve bile flow. Primary sclerosing cholangitis is, in fact, one of the more common reasons for liver transplantation. The treatment of primary sclerosing cholangitis includes:

  • ERCP with balloon dilatation and/or stenting. This is when the bile ducts are stretched open for primary sclerosing cholangitis patients with dominant extra-hepatic duct strictures
  • Antibiotics for infection, specifically cholangitis
  • Medications such as cholestyramine (Questran) or rifampin (Rifadin) to diminish itching
  • Vitamin D and calcium to prevent bone loss (osteoporosis)
  • Medications such as ursodiol (UDCA) to delay the progression of primary sclerosing cholangitis
  • For advanced cirrhosis a liver transplant may be necessary.

PSC can lead to various complications such as:

  • vitamins A, D, E, and K deficiencies
  • liver failure
  • bile duct cancer
  • infections of the bile ducts
  • cirrhosis of the liver

PSC has been known for 100 years, but now doctors are able to diagnose it very early.  Treatment can begin before the liver is badly damaged. Researchers and scientists continue to study PSC to find the cause and cure.

Primary sclerosing-cholangitis patients are seen in our Poughkeepsie, Kingston, and New Windsor offices.