DISEASE

Primary Sclerosing Cholangitis

OVERVIEW | CAUSES | RISK FACTORS | SYMPTOMS | COMPLICATION | DIAGNOSIS | TREATMENT | PREVENTION | REFERENCES

OVERVIEW

Primary sclerosing cholangitis 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. A majority of people with primary sclerosing cholangitis also have inflammatory bowel disease, such as ulcerative colitis or Crohn's disease.

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.

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, 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.

RISK FACTORS

Factors that may increase the risk of primary sclerosing cholangitis include:

Age. Primary sclerosing cholangitis can occur at any age, but it's most often diagnosed between the ages of 30 and 40.
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 with Northern European heritage have a higher risk of primary sclerosing cholangitis.

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 signs and symptoms often include:

Fatigue
Itching
Yellow eyes and skin (jaundice)
Abdominal pain

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:

Fever
Chills
Night sweats
Enlarged liver
Enlarged spleen
Weight loss

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, 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.

DIAGNOSIS

Tests and procedures used to diagnose primary sclerosing cholangitis include:

Liver function blood test. 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 and is the test of choice to diagnose primary sclerosing cholangitis.
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.
Liver biopsy. 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.

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

Bile acid sequestrants. 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.
Antibiotics. 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.
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.
Opioid antagonists. 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.
Ursodeoxycholic acid (UDCA). Also known as ursodiol, UDCA is a naturally occurring bile acid that may help relieve itching symptoms caused by liver disease by increasing the absorbability of bile.

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 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:

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.

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.

PREVENTION

Since the exact cause for primary sclerosing cholangitis cannot be identified, there are no proven preventive measures.