Primary Sclerosing Cholangitis

Cholangitis is an inflammation of the bile ducts. This condition usually heals quickly with treatments. If the condition becomes chronic and involves the liver, it is known as primary sclerosing cholangitis. Sclerosing cholangitis means that the bile ducts and liver become damaged with scarring. The condition is rare, but 1 in 10,000 people are diagnosed with primary sclerosing cholangitis in the U.S. each year. It is mostly found in men around the age of 40, but can also occur in women.

What Is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis results from long-term inflammation of the bile ducts that causes scarring and damage. The bile ducts carry bile from the liver to the small intestine to help digest the food we eat and help our body absorb vitamins. When someone has PSC, the scarring causes blockage so the bile backs up into the liver. This in turn causes scarring on the liver and liver damage.

What Are the Symptoms & Complications of Primary Sclerosing Cholangitis?

Most of the time, doctors find this condition before the person even has any symptoms. This is due to abnormal liver tests or an abnormal liver x-ray that have been done for other reasons. If there are early symptoms, they usually include:

• Itchy skin
• Feeling tired

The disease can be present for many years, but as the disease advances the symptoms range from:

• Fevers and chills
• Night sweats
• Unexplained weight loss
• Right upper abdominal pain
• Liver enlargement
• Jaundice (yellowing of the skin/eyes)

Complications

Because this disorder deals with the digestive system and function of the liver, complications include:

• Vitamin deficiencies
• Bile duct infection
• Cirrhosis of the liver
• Liver failure
• Cancer of the bile duct

When to See a Doctor

If you begin to feel severe itching all over your body that does not go away, you should contact your doctor. You should also see your doctor if you experience fatigue that does not go away. Also, if you have any type of inflammatory bowel disease along with these symptoms, you should contact your doctor. Evidence has been found that people with ulcerative colitis or Crohn’s disease may be more susceptible to primary sclerosing cholangitis.

What Are the Causes of Primary Sclerosing Cholangitis?

Researchers have not pinpointed the exact cause, but there are a few factors that may be related to getting PSC. These include:

• 70 percent of sufferers are male
• Onset between the ages of 30 to 60
• 75 percent of sufferers have either Crohn’s or ulcerative colitis
• Genetics may play a role
• Possible viral or bacterial infection prior to onset
• There are more people in the U.S. and Northern Europe with this condition than Asia or Southern European countries

What Are the Treatments for Primary Sclerosing Cholangitis?

While there are no medications that treat the actual condition, doctors aim treatments at controlling symptoms and preventing complications. At this time there are no medications that can reverse any liver damage that has already occurred. Here are a few of the treatments used to manage the symptoms:

• For itching – There is a medication known as cholestyramine that can help relieve skin itching. If this is not effective, the doctor may try other medications to relieve itching.
• Vitamin absorption – Sometimes vitamin supplementation is needed to help reduce malnutrition. Vitamins replaced are the fat-soluble vitamins A, D, E, and K.
• Bile flow and digestion – Ursodeoxycholic acid may help increase bile flow and aid digestion. This medication can also increase liver function.
• Infection – If there is acute cholangitis present, the doctor will prescribe antibiotics to control infection.
• Scarring and obstruction – The doctor may order a cholangiogram to look for a bile duct blockage. They can stent the duct to help drain the bile.
• Diet – Those with liver related disease do not absorb proteins as well as they should. Diet needs to be protein and calorie rich, including extra fats.
• No alcohol – Alcohol consumption needs to be stopped completely or only minute amounts taken in. Alcohol can increase inflammation in the liver and add to scarring and cirrhosis.
• When Do Patients Need Liver Transplants?

Liver complications may eventually lead to complete liver failure. In this case, patients may need a liver transplant. After transplantation, there is usually an 80 percent survival rate and improvement of quality of life. After liver transplant, here are some statistics out of 17 patients that received liver transplants:

• 15 out of 17 patients only required one liver transplant
• 2 of those patients needed a second liver transplant
• 2 patients developed bile cancer and the outcome was fatal
• 2 patients developed recurrent sclerosing cholangitis

What Is the Prognosis for Primary Sclerosing Cholangitis?

The prognosis for primary sclerosing cholangitis depends on the case. People who don’t suffer from any symptoms of the disease have a better prognosis than those with symptoms. The cancer rate is high with about 1 out of 10 people with primary sclerosing cholangitis developing bile duct cancer at some point during the course of the disease. This type of cancer is very hard to treat. There is ongoing research to develop good screening methods for early detection of bile duct cancer. A small patient population also develops cancer of the colon with primary sclerosing cholangitis. After liver transplantation, the prognosis is very good if the patient does not develop a second case of primary sclerosing cholangitis or bile duct cancer.

Primary sclerosing cholangitis is generally a new and developing field of medicine and research is ongoing. It is important to find a knowledgeable specialist that can help with the latest treatments and more information on this disease.