Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) 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 liver failure, repeated infections, and tumors of the bile duct or liver. Liver transplant is the only known cure for primary sclerosing cholangitis.

The search for other treatments to slow or stop primary sclerosing cholangitis is ongoing, and scientists have turned up many promising leads. Until better treatments are proved safe and effective, though, care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.

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
  • Itching

Many people diagnosed with primary sclerosing cholangitis before they have symptoms continue to feel generally well for several years, but there's 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
  • Yellow eyes and skin (jaundice)

When to see a doctor

Make an appointment with your doctor if you have severe, unexplained itching on much of your body — itching that persists no matter how much you scratch. Also see your doctor if you feel extremely tired all the time, no matter what you do.

It's particularly important to bring unexplained fatigue and itching to your doctor's attention if you have ulcerative colitis or Crohn's disease, both of which are types of inflammatory bowel disease. A majority of people with primary sclerosing cholangitis also have one of these diseases.

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, an umbrella term that includes ulcerative colitis and Crohn's disease. Primary sclerosing cholangitis and inflammatory bowel disease don't 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, people being 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.

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.
  • Your 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.

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, loss of liver function.
  • Repeated infections. If scarring of the bile ducts slows or stops the flow of bile out of the liver, you may experience frequent infections in the bile ducts. The risk of infection is particularly high after you've had a surgical procedure to expand a badly scarred bile duct or remove a stone blocking a bile duct.
  • 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.

Start by seeing your primary care doctor 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. When you make your 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 remember 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. You may feel less rushed if you make a list of questions you want to ask at your appointment. Here are some possible questions about primary sclerosing cholangitis:

  • 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?
  • 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?
  • Do you have frequent bouts of diarrhea?
  • Have you noticed blood in your stool?

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.

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 unknown. 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.

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 infections

Bile that backs up in narrowed or blocked ducts causes frequent 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. Before any procedure that could cause an infection, such as an endoscopic procedure or abdominal surgery, you'll also need to take antibiotics.

Nutrition support

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. If the disease weakens your bones, you may take calcium and vitamin D supplements as well.

Treatment for bile duct blockages

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.
  • 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.

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.

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.
  • 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.

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
  • Experience. Mayo Clinic is a leading treatment center for people with primary sclerosing cholangitis. The Transplant Center at Mayo Clinic has the largest liver transplant program in the United States and has performed many transplants in people who have primary sclerosing cholangitis. Mayo Clinic is one of the few medical centers in the country that treats bile duct cancer with a liver transplant.
  • Expertise. Liver specialists at Mayo Clinic have developed several mathematical models that assess the severity of certain liver diseases. These models help doctors determine where a person ranks on the priority list for a transplant.
  • Team approach. Effective treatment of primary sclerosing cholangitis requires coordinated care by several specialties. Liver specialists (hepatologists), endoscopy specialists, pathologists, oncologists, pediatric specialists and surgeons work as a team to provide comprehensive care for adults and children with primary sclerosing cholangitis.
  • Continuous improvement. Many Mayo Clinic doctors are involved in clinical trials of new medications for primary sclerosing cholangitis. Researchers at Mayo Clinic are also studying the DNA of people with primary sclerosing cholangitis in hopes of identifying genes that increase the risk of the disease and predict its course.
  • Liver transplant. Mayo Clinic transplant specialists have performed many transplants in people who have primary sclerosing cholangitis. People with primary sclerosing cholangitis who have liver transplants generally have high survival rates — 80 percent after five years on average. Recipients of liver transplants at Mayo Clinic have above-average survival rates.

    Mayo Clinic is also one of the few medical centers in the country to offer liver transplant as a treatment option for bile duct cancer, which develops at much higher rates among people with primary sclerosing cholangitis than in the general population. A multidisciplinary team of Mayo Clinic hepatologists, surgical specialists, radiation oncologists and medical oncologists follows a several-stage treatment plan to prepare people with bile duct cancer for liver transplantation and provide comprehensive medical services during recovery and follow-up.

Mayo Clinic in Rochester, Minn., ranks #1 for digestive disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., is ranked among the Best Hospitals, and Mayo Clinic in Jacksonville, Fla., is ranked high performing for digestive disorders by U.S. News & World Report. Mayo Clinic also ranks among the Best Children's Hospitals for digestive disorders.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

Specialists in gastroenterology and hepatology at Mayo Clinic's campus in Arizona coordinate the diagnosis and treatment of primary sclerosing cholangitis. A special Hepatobiliary Clinic helps coordinate medical care as well as research on new treatments. The Arizona campus also has an active liver transplant program.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

Specialists in gastroenterology and hepatology at Mayo Clinic's campus in Florida coordinate the diagnosis and treatment of primary sclerosing cholangitis. The liver transplant program in Florida is a national leader in the number of liver transplants performed. The liver transplant program has one of the shortest wait times to transplant in the country.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

At Mayo Clinic's campus in Minnesota, specialists in gastroenterology and hepatology diagnose and treat people who have primary sclerosing cholangitis. These specialists also work with transplant surgeons and other experts to provide a seamless patient experience in the Mayo Clinic Transplant Center. A Hepatobiliary Clinic coordinates medical care with research.

Specialists in pediatric hepatology at Mayo Clinic's campus in Minnesota are experienced in accurately diagnosing children who have liver diseases.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Mayo Clinic researchers were among the first to describe primary sclerosing cholangitis. Other research done at Mayo has helped to refine diagnostic tests, improve treatment and assess the results of liver transplants for people with primary sclerosing cholangitis. Mayo Clinic researchers also developed mathematical models to help physicians predict long-term survival for people with primary sclerosing cholangitis.

Researchers at Mayo Clinic are currently studying the use of the fluorescence in situ hybridization (FISH) test in the diagnosis of bile duct cancer in people with primary sclerosing cholangitis. Another study is looking at genetic predisposition to primary sclerosing cholangitis.

Basic research related to primary sclerosing cholangitis and other liver diseases is conducted in the following Mayo Clinic laboratories:

Publications

See a list of publications by Mayo Clinic doctors on primary sclerosing cholangitis on PubMed, a service of the National Library of Medicine.

Aug. 15, 2014