Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disease of the bile ducts, which carry the digestive liquid, bile, from your liver to your small intestine. In primary sclerosing cholangitis, inflammation causes scars within the bile ducts. These scars make the ducts hard and narrow and gradually cause serious liver damage. In most people with primary sclerosing cholangitis, the disease progresses slowly and can lead to cirrhosis, liver failure, repeated infections, and tumors of the bile duct or liver.

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 (IBD), an umbrella term that includes Ulcerative Colitis and Crohn's disease.

Primary sclerosing cholangitis and inflammatory bowel disease do not 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, those 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.
  • 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.

Signs and 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 and itching. Many people diagnosed with primary sclerosing cholangitis generally feel well for years, but there is 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 and yellow eyes and skin (jaundice).

Screening and Diagnostic Tests

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.

Treatment

Treatments for primary sclerosing cholangitis focus on managing complications and monitoring liver damage. Many medications have been studied in people with primary sclerosing cholangitis, but so far none have been found to slow or reverse the liver damage associated with this disease.

  • Treatment for Itching. Antihistamines, such as diphenhydramine (Benadryl, Diphenhist, others) and loratadine (Alavert, Claritin, others), are commonly used to reduce itching from insect bites or contact dermatitis. Whether these medications are effective in primary sclerosing cholangitis, though, is varied. Antihistamines may worsen the liver disease symptoms of dry eyes and dry mouth. On the other hand, antihistamines can help with sleep if itching keeps you awake. Medications that bind to bile acids — the substances thought to cause itching in liver disease — are the first line treatment for itching in primary sclerosing cholangitis (eg cholestyramine, colestipol). If you have trouble tolerating a bile acid-binding drug or if it doesn't help, your doctor may prescribe rifampin (Rifadin, Rimactane, others), an antibacterial drug. Exactly how rifampin reduces itching is unknown, but it may block the brain's response to itch-inducing chemicals in your circulation. Itching related to liver disease may also respond to opioid antagonist drugs, such as naltrexone. Like rifampin, these drugs seem to reduce the itch sensation by acting on your brain.
  • Treatment for Infection. Bile that backs up in narrowed or blocked ducts may cause bacterial infections. To prevent and treat these infections, people with primary sclerosing cholangitis may take repeated courses of antibiotics or continue taking antibiotics for long periods. You will also need to take antibiotics before undergoing any procedure that could cause an infection, such as an ERCP endoscopic procedure or abdominal surgery.
  • Nutrition Support. Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins (A, D, E, K). Even though you may eat a healthy diet, you may find that you can't get all the nutrients you need. Your doctor will usually recommend vitamin supplements that you take as tablets or (rarely) that you receive as an infusion through a vein in your arm. If the disease weakens your bones, you may take calcium and vitamin D supplements as well; vitamin D level is monitored and a type of bone scan (DEXA) is periodically done to monitor for bone health and guide treatment

Treatment for Bile Duct Blockage

Blockages that occur in your bile ducts can be treated with:

  • Balloon dilation. This procedure can open blockages in the larger bile ducts outside the liver. In balloon dilation, your doctor runs a slender tube with an inflatable balloon at its tip (balloon catheter) through an endoscope and into a blocked bile duct. Once the balloon catheter is in place, the balloon is inflated. This is seldom needed and does not change the underlying disease.
  • Stent placement. In this procedure, your doctor uses an endoscope and attached instruments to place a small plastic tube called a stent in a blocked bile duct to hold the duct open. This is seldom needed and does not change the underlying disease.
  • Liver Transplant. A liver transplant is the only treatment known to cure primary sclerosing cholangitis. During a liver transplant, surgeons remove your diseased liver and replace it with a healthy liver from a donor. Liver transplant is reserved for people with liver failure or other severe complications of primary sclerosing cholangitis. Though uncommon, it's possible for primary sclerosing cholangitis to recur after a liver transplant. We refer patients to liver transplant centers LONG before we believe this treatment is necessary but when we can foresee that it MAY become life-saving. Careful assessment and regular follow-up are done when individuals are candidates for transplant, so the timing of this can be optimal and the patient and family well prepared to deal with the complexity of liver.

Lifestyle and Home Remedies

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

  • Avoid consumption of 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.