Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) is a disease of the bile ducts. Bile ducts 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. It can eventually lead to liver failure, repeated infections, and tumors of the bile duct or liver. A liver transplant is the only known cure for advanced primary sclerosing cholangitis, but the disease may recur in the transplanted liver in a small number of patients.
Care for primary sclerosing cholangitis focuses on monitoring liver function, managing symptoms and, when possible, doing procedures that temporarily open blocked bile ducts.
The bile ducts carry bile from your liver to your small intestine. When bile ducts become damaged, bile can back up into the liver, causing damage to liver cells. This damage can lead to liver failure.
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:
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:
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. If primary sclerosing cholangitis is diagnosed, it's important to look for inflammatory bowel disease because there is a greater risk of colon cancer.
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:
Complications of primary sclerosing cholangitis may include:
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.
Tests and procedures used to diagnose primary sclerosing cholangitis include:
X-rays of your bile ducts. A type of bile duct X-ray called endoscopic retrograde cholangiopancreatography (ERCP) in addition to, or instead of, an MRI may be needed. But this test is rarely used for diagnosis because of the risk of complications.
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.
An ERCP is the test of choice if signs and symptoms persist despite no abnormalities on an MRI. An 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.
A 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.
Endoscopic retrograde cholangiopancreatography (ERCP) uses a dye to highlight the bile ducts on X-ray images. A thin, flexible tube (endoscope) with a camera on the end is passed down your throat and into your small intestine. The dye enters the ducts through a small hollow tube (catheter) passed through the endoscope.
A liver biopsy is a procedure to remove a small sample of liver tissue for laboratory testing. A liver biopsy is commonly performed by inserting a thin needle through your skin and into your liver.
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.
Antihistamines. This type of medication may help reduce mild itching caused by primary sclerosing cholangitis. Whether these medications are effective for this condition 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.
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.
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.
Blockages that occur in your bile ducts may be due to disease progression but can be a sign of cancer of the bile duct. Endoscopic retrograde cholangiopancreatography (ERCP) can help determine the cause, and bile duct blockage can be treated with:
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.
A 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:
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:
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.
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:
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.
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: