Mayo Clinic Health Library

Primary sclerosing cholangitis

Updated: 04-09-2011

Definition

Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) is a disease of the bile ducts in your liver. The term "cholangitis" in primary sclerosing cholangitis refers to inflammation of the bile ducts, while the term "sclerosing" describes the hardening and scarring of the bile ducts that result from chronic inflammation.

Primary sclerosing cholangitis is a progressive disease that leads to liver damage and, eventually, liver failure. Liver transplant is the only known cure for primary sclerosing cholangitis, but transplant is typically reserved for people with severe liver damage.

Researchers continue looking for treatments to slow or reverse bile duct damage caused by primary sclerosing cholangitis. But until a treatment is found, doctors care for people with primary sclerosing cholangitis by reducing signs and symptoms of complications.

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Symptoms

Signs and symptoms of primary sclerosing cholangitis include:

  • Abdominal pain
  • Chills
  • Diarrhea
  • Fatigue
  • Fever
  • Itchiness
  • Weight loss
  • Yellowing of your eyes and skin (jaundice)

Primary sclerosing cholangitis may not cause any symptoms in its early stages. In some cases, the only indication of this disorder may be abnormal blood tests suggesting that your liver isn't functioning well.

When to see a doctor
Make an appointment with your doctor if you have any persistent signs and symptoms of primary sclerosing cholangitis.

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Causes

It's not clear what causes primary sclerosing cholangitis. It's believed that the condition may be caused by an immune system reaction to an infection or toxin in people with a predisposition to develop the disease.

Primary sclerosing cholangitis occurs more frequently in people with certain other medical conditions, particularly inflammatory bowel disease. It's not clear how these conditions are linked and whether they share similar causes.

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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 commonly diagnosed in people ages 25 to 45.
  • Your sex. Primary sclerosing cholangitis occurs more often in men than it does in women.
  • Inflammatory bowel disease. The great majority of people with primary sclerosing cholangitis also have inflammatory bowel disease, which includes ulcerative colitis and Crohn's disease. Still, primary sclerosing cholangitis is rare among people with inflammatory bowel disease, and most won't develop primary sclerosing cholangitis. It's not clear why these diseases occur together. If you've been diagnosed with primary sclerosing cholangitis, your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms.
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Complications

Complications of primary sclerosing cholangitis may include:

  • Liver disease and failure. Chronic inflammation of the bile ducts throughout your liver can lead to tissue scarring (cirrhosis), liver cell death and, eventually, the inability of your liver to function properly.
  • Repeated infections. If scarring of the bile ducts impairs the flow of bile out of the liver, you may experience frequent infections in the bile ducts.
  • Portal hypertension. Your portal vein is the major route for blood flowing from your digestive system into your liver. Portal hypertension refers to high blood pressure in this vein. Portal hypertension can cause fluid from the liver to leak into your abdominal cavity (ascites). It can also divert blood from the portal vein to other veins, causing these veins to become swollen (varices). Varices are weak veins and tend to bleed easily, which can be life-threatening.
  • Thinning bones. People with primary sclerosing cholangitis may experience thinning bones (osteoporosis). Your doctor may recommend a bone density exam to test for osteoporosis every few years. Calcium and vitamin D supplements may be prescribed to help prevent bone loss.
  • Bile duct cancer. If you have primary sclerosing cholangitis, you have an increased risk of developing cancer in the bile ducts or gallbladder.
  • Colon cancer. People with primary sclerosing cholangitis associated with inflammatory bowel disease have an increased risk of colon cancer. If you've been diagnosed with primary sclerosing cholangitis, your doctor may recommend testing for inflammatory bowel disease, even if you have no signs or symptoms, since the risk of colon cancer is elevated if you have both diseases.
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Preparing for your appointment

Start by seeing your family doctor or a general practitioner if you have signs or symptoms that worry you. If your doctor suspects you may have primary sclerosing cholangitis, you may be referred to a liver specialist (gastroenterologist or hepatologist).

Because appointments can be brief, and because there's often a lot of ground to cover, it's a good idea to be well prepared. Here's some information to help you get ready, and what to expect from your doctor.

What you can do

  • Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
  • Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
  • Write down key personal information, including any major stresses or recent life changes.
  • Make a list of all medications, as well as any vitamins or supplements, that you're taking.
  • Consider taking a family member or friend along. Sometimes it can be difficult to absorb all the information provided during an appointment. Someone who accompanies you may remember something that you missed or forgot.
  • Write down questions to ask your doctor.

Your time with your doctor is limited, so preparing a list of questions will help you make the most of your time together. List your questions from most important to least important in case time runs out. For primary sclerosing cholangitis, some basic questions to ask your doctor include:

  • Can you explain my test results to me?
  • Will I need more tests?
  • How far has my primary sclerosing cholangitis progressed?
  • How severe is the damage to my liver?
  • Can you estimate when I may need a liver transplant?
  • What treatments can relieve my signs and symptoms?
  • What are the potential side effects of each treatment?
  • Should I be tested for inflammatory bowel disease?
  • What signs and symptoms signal that my condition is worsening and I need to make another appointment?
  • Are there any restrictions that I need to follow?
  • Should I see a specialist? What will that cost, and will my insurance cover it?
  • Are there any brochures or other printed material that I can take with me? What websites do you recommend?

In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask questions that come up during your appointment.

What to expect from your doctor
Your doctor is likely to ask you a number of questions. Being ready to answer them may allow more time later to cover points you want to address. Your doctor may ask:

  • When did you begin experiencing symptoms?
  • Have your symptoms been continuous or occasional?
  • How severe are your symptoms?
  • What, if anything, seems to improve your symptoms?
  • What, if anything, appears to worsen your symptoms?
  • Have you experienced episodes of prolonged diarrhea?
  • Have you noticed blood in your stool?
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Tests and diagnosis

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.
  • X-rays of your bile ducts. Injecting a dye into your bile ducts helps make them visible on an X-ray. To inject the dye, your doctor may recommend endoscopic retrograde cholangiopancreatography. During this procedure, your doctor guides a flexible tube down your throat and through your stomach to the area of your small intestine where your bile ducts empty. Using the tube and special tools, your doctor injects dye into the bile ducts.
  • MRI of your bile ducts. Magnetic resonance cholangiopancreatography uses magnetic resonance imaging (MRI) to make images of your liver and bile ducts.
  • 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.
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Treatments and drugs

Treatments for primary sclerosing cholangitis focus on reducing signs and symptoms of the disease as it progresses. Primary sclerosing cholangitis progresses slowly, but it usually ends in liver failure and the need for a liver transplant. Many medications have been studied in people with primary sclerosing cholangitis, but so far none has been proved to slow or reverse the liver damage associated with this disease.

Treatment for itching
Medications that may help control itching include:

  • Antihistamines, such as diphenhydramine (Benadryl, others)
  • Bile-acid-binding drugs, such as cholestyramine (Locholest, Questran, others)

Treatment for infections
Bacterial infections can occur frequently in people with primary sclerosing cholangitis. Antibiotics may be prescribed to treat an infection. If you've had repeated infections, your doctor may recommend antibiotics before procedures that are likely to cause infection, such as endoscopic procedures or surgery.

Treatment for malnutrition
Primary sclerosing cholangitis makes it difficult for your body to absorb certain vitamins. Even though you may eat a healthy diet, you may find that you can't get all the nutrients you need. Your doctor may recommend vitamin supplements that you take as tablets or that you receive as an infusion through a vein in your arm.

Treatment for bile duct blockages
Blockages that occur in your bile ducts can be treated with:

  • Balloon dilation and stent placement. These procedures can open blockages in the larger bile ducts. Balloon dilation is a procedure in which 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. Small plastic tubes called stents may be placed in bile ducts to keep them open.
  • Bile duct surgery. In certain situations, blockages in bile ducts may need to be removed surgically. After removing a blockage, the surgeon connects the remaining portions of bile duct so that bile can still flow through the duct.

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.

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Lifestyle and home remedies

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

  • Don't drink alcohol.
  • Get vaccinated against hepatitis A and B.
  • Use care with chemicals at home and at work.
  • Quit smoking. If you don't smoke, don't start.
  • Choose a healthy diet full of fruits, vegetables and whole grains.
  • Maintain a healthy weight.
  • Follow directions on over-the-counter medications.
  • Talk to your doctor about any herbs or supplements you're taking, since some can be harmful to your liver.
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Alternative medicine

No alternative medicine treatments have been found to treat primary sclerosing cholangitis. But some complementary and alternative therapies may help you cope with the signs and symptoms of the disease. Talk to your doctor about your options.

Fatigue is common in people with primary sclerosing cholangitis. While doctors can treat some factors that may contribute to fatigue, your signs and symptoms may still persist. You might find relief with complementary and alternative treatments that have shown some benefit for fatigue, such as:

  • Exercise, such as walking 30 minutes most days of the week
  • Spending time with friends and family
  • Stress management techniques, such as meditation and relaxation exercises
  • Yoga
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