Primary Sclerosing Cholangitis – Causes, Symptoms and Treatment

What is Primary Sclerosing Cholangitis?

Primary sclerosing cholangitis (PSC) is a condition that damages the bile ducts. In this disease, inflammation causes scars within the bile ducts. The scars make the bile ducts hard and narrow and slowly lead to serious liver damage. Most of the PSC patients also have inflammatory bowel disease, such as ulcerative colitis or Crohn’s disease. The bile ducts carry bile, which is a digestive liquid that helps in the digestion of fats and fatty acids. In the majority of people, the disease progression is slow but serious cases can lead to liver failure.

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What are the Causes?

An immune system reaction to a toxin or infection might trigger the disease in individuals genetically predisposed to it.

Most people with primary sclerosing cholangitis also have inflammatory bowel disease, an umbrella term which includes Crohn’s disease and ulcerative colitis.

What are the Symptoms of Primary Sclerosing Cholangitis ?

Many people continue to feel generally well for several years. It is difficult predict how quickly or slowly the disease will progress for any individual.

Early symptoms

• Itching on the body
• Yellow eyes and skin due to onset of jaundice
• Abdominal pain
• Fatigue

The symptoms that arise when the disease progresses are

1. Enlargement of liver and spleen
2. Fever
3. Loss of weight
4. Fever with chills
5. Night sweats 

When to see a doctor?

You must meet your doctor if you have any of the signs and symptoms mentioned above. Also itching and fatigue that refuses to go away are early signs that need medical evaluation .

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What are the Risk Factors?

The risk factors that are associated with the disease are,

• Inflammatory bowel disease: many people with primary sclerosing cholangitis also have inflammatory bowel disease.
• Sex of the patient: it has been observed that the occurrence of the disease is more common in men than in women.
• Age group: It can occur during any age but is most commonly seen in people with age groups ranging from 30 to 40 years.
• Location: risk of contracting the disease is much higher in people having Northern European Heritage.

What are the Complications?

The complications associated with it include:

1. Liver failure: chronic cases of primary sclerosing cholangitis lead to permanent blockage of the bile duct. This in turn leads to cirrhosis of the liver and liver failure.
2. Portal hypertension: the portal veins help in blood flow from the digestive system to the liver. Portal hypertension happens when there is high blood pressure in this vein. This causes leakage of fluid from the liver into the abdominal cavity and swollen veins. The swollen veins, also known as varices, often bleed easily and can lead to life-threatening conditions.
3. Colon cancer: primary sclerosing cholangitis along with inflammatory bowel disease leads to increased risk of colon cancer.
4. Bile duct cancer: primary sclerosing cholangitis also increases the risk of developing cancer of the bile ducts and the gallbladder.
5. Repeated infections: If the scarring of bile ducts stops or slows the flow of bile out of your liver, you may experience frequent infections in the bile ducts
6. Thinning bones or osteoporosis 

How is it Diagnosed? 

Tests and techniques involved in the diagnosis of primary sclerosing cholangitis are as below:

• MRI of the bile duct: MRI of the bile ducts and the liver is done to obtain images that help in the detection of primary sclerosing cholangitis.
• X-ray of the bile duct: Also known as endoscopic retrograde cholangiopancreatography (ERCP), is done for the initial diagnosis of primary sclerosing cholangitis and is usually done in cases where MRI cannot be done.
• Blood tests: Blood tests are performed to determine the liver function, which can give the doctor a clear idea about the occurrence of primary sclerosing cholangitis.
• Liver biopsy: to carry out a liver biopsy, a small piece of liver tissue is obtained . The liver biopsy also helps to determine the extent of liver damage.

What are the Treatments of Primary Sclerosing Cholangitis?

Treatments include medication that can reduce complications and subsequent liver damages. A few of the treatment strategies include:

Treating itching

Itching can be treated in the following ways:

• Antibiotics: Rifampicin often helps to reduce itching by blocking the brain’s response to itch-inducing chemicals. 
• Antihistamines: antihistamines are used to treat primary sclerosing as they help to control itching effectively.
• Bile acid sequestrants: bile acid sequestrants are substances that bind with the bile acid, which is known to cause itching and thus helps to treat primary sclerosing cholangitis.
• Ursodeoxycholic acid (UDCA): UDCA is a naturally-occurring bile acid that increases the absorbability of bile and thus reduces itching.

Treating bile duct blockages

Bile duct blockages can be treated by the following methods:

• Stent placement: this technique uses an endoscope along with attached instruments to place a small plastic tube known as A stent in a blocked bile duct, which helps in opening the duct.
• Balloon dilation: this technique uses an endoscope with a slender tube having an inflated balloon at its tip. This is then placed into the blocked bile duct and the inflated balloon causes the bile duct to open.
• Liver transplant: it is done in chronic cases of primary sclerosing cholangitis. The diseased liver is replaced by a healthy liver that allows the patient to go back to leading a normal life.

Conclusion

Primary sclerosing cholangitis is a disease that causes the blockage of the bile ducts. The symptoms of the disease are varied. The disease can be detected by many techniques such as MRI, X-ray, biopsy of the liver and blood tests. The treatment of the disease usually involves treating the symptoms and the complications associated with the disease.

Frequently Asked Questions (FAQs) 

How long can you survive with primary sclerosing cholangitis?

The progression of primary sclerosing cholangitis is slow. As a result, chronic symptoms arise slowly over a period of 10 to 15 years.

Does alcohol play any role in Primary Sclerosing Cholangitis?

No correlation was found between alcohol and the development of Primary Sclerosing Cholangitis.

What should you avoid eating during Primary Sclerosing Cholangitis?

Doctors recommend patients avoid having raw shellfish that may harbor bacteria. Along with it, doctors also prescribe foods that are low in fats, carbohydrates, and salts during the disease.