A condition of the bile ducts in which inflammation causes scars within the bile ducts is called primary sclerosing cholangitis. These ducts carry the digestive liquid (bile) from the liver to the small bowel. Scars that appear due to inflammation may narrow the ducts and make it difficult for bile to flow. Over time, this condition causes serious damage to the liver. Roughly all people with this disease also have inflammatory bowel disease, such as Crohn’s disease, ulcerative colitis, or another one.
Usually, this bile duct condition progresses slowly. Without treatment, it may lead to liver failure, recurrent infections, and tumors of the bile duct or liver. The only way to cure this condition is with a liver transplant. However, the condition may return in some people.
In mild cases, treatment focuses on monitoring liver function and managing the symptoms, and sometimes doctors may recommend some therapies to open blocked bile ducts.
Symptoms
Mostly, primary sclerosing cholangitis is diagnosed before the symptoms appear during a routine blood test, X-ray, or another test. Check below some early symptoms of this condition:
Those who suffer from this condition without symptoms feel well for years. The following symptoms often appear when the disease advances. For example:
- Fever
- Night sweats
- Enlarged liver or spleen
- Weight loss
- Chills
If you experience severe itching on the body without any obvious reason, immediately visit a healthcare professional. Inform your doctor if you experience unexplained extreme tiredness and intense itching, especially if you experience ulcerative colitis, Crohn’s disease, or another inflammatory bowel disease. Most people with primary sclerosing cholangitis have one of the previous conditions.
Causes
Healthcare providers do not fully understand why primary sclerosing cholangitis happens. However, an immune system reaction to an infection may trigger the disease in people predisposed to it. Furthermore, most people with this disease also have an inflammatory bowel disease.
In any case, inflammatory bowel diseases and primary sclerosing cholangitis do not appear at the same time usually. For instance, a person may have primary sclerosing cholangitis for years before an inflammatory bowel disease. People who are diagnosed with primary sclerosing cholangitis also should be tested for inflammatory bowel disease because there is an elevated risk of colon cancer.
In rare cases, those who are treated for an inflammatory bowel disease develop primary sclerosing cholangitis. Moreover, some people may develop inflammatory bowel diseases only after a liver transplant, but this occurs very rarely.
Risk Factors
There are some factors that may elevate your risk of developing primary sclerosing cholangitis. For example:
- Age – While it may happen at any age, it mostly is diagnosed in people between the ages of 30 and 40.
- Sex – Males are more prone to develop this bile duct condition.
- Inflammatory bowel disease – Most people with primary sclerosing cholangitis also have an inflammatory bowel disease.
- Geographical location – An increased risk of developing primary sclerosing cholangitis exists in people with Northern European heritage.
What Are The Potential Complications of Primary Sclerosing Cholangitis?
People with this condition may experience some complications, especially if they do not get treatment. Check below some examples:
- Liver disease and failure – Long-term (chronic) inflammation of the bile ducts may lead to scars on the liver (cirrhosis), liver cell death, as well as loss of liver function.
- Recurrent infections – When the bile ducts become clogged, it may lead to frequent infections. The risk of infections also increases if you have surgeries to open the bile ducts.
- Portal hypertension – This high blood pressure type happens when it occurs in the portal vein (a major route for blood flowing from the digestive system into the liver). Furthermore, this hypertension type may make the liver fluid leak into the abdominal cavity (ascites). This complication may also divert the blood from this vein and cause swelling of the veins (varices). This is a life-threatening condition.
- Osteoporosis – This is a condition in which the bones become weak. People with osteoporosis are at increased risk of bone fractures. In such cases, physicians usually recommend calcium and vitamin D supplements.
- Bile duct cancer – The risk of bile duct cancer significantly increases in people with primary sclerosing cholangitis.
- Colon cancer – The risk of colon cancer increases in people with this disease because they also have inflammatory bowel disease. In people with both conditions, the risk of this complication significantly increases.
Diagnosis
Doctors usually perform the following tests to diagnose this bile duct condition. Examples include:
- Blood tests – These tests are performed by doctors to check liver function (including increased levels of liver enzymes that may indicate the disease).
- MRI of the bile ducts – This is an imaging test used to get detailed images of the liver and bile ducts. It is called magnetic resonance cholangiopancreatography.
- X-rays of the bile ducts – It is another imaging test (also called endoscopic retrograde cholangiopancreatography or ERCP) that can be used in addition to or instead of MRI. However, it is rarely used to diagnose primary sclerosing cholangitis because of an increased risk of complications. During this test, doctors will inject a dye that will help make bile ducts visible on an X-ray.
- Liver biopsy – This test involves the removal of a small piece of the liver and testing it in the laboratory. It helps determine the extent of liver damage and to confirm the disease.
Treatment
The treatment goal is to lessen the symptoms, prevent complications, and monitor liver damage. Despite multiple studies, there are no medicines that may stop or reverse liver damage in people with primary sclerosing cholangitis. Check below some treatments often recommended by doctors:
Treatment for Itching
- Bile acid sequestrants – This is the primary treatment for itching caused by primary sclerosing cholangitis. These medications work by binding to bile acids (substances in the liver that cause itching).
- Antibiotics – This group of medicines is recommended to reduce itching and block the brain’s response to itch-inducing chemicals. In most cases, physicians prescribe Rifampin.
- Antihistamines – These medicines may help with mild itching in people with primary sclerosing cholangitis. On the other hand, antihistamines may worsen liver disease symptoms. Discuss with your healthcare professional before taking them.
- Opioid antagonists – Commonly, doctors prescribe Naltrexone to lessen itching linked with liver disease.
- Ursodeoxycholic acid (UDCA) – It is also called Ursodiol, and it is a bile acid that occurs naturally and helps lessen itching symptoms.
Treatment for Infections
Recurrent bacterial infections may occur when bile ducts are narrowed or clogged. Thus, physicians may recommend some medicines to treat infections. They usually prescribe antibiotics that often are taken for long periods.
Nutrition Support
This disease makes it challenging for the body to absorb nutrients, even if you have a healthy diet. Therefore, doctors may recommend supplements to ensure proper nutrition.
Treatment for Bile Duct Blockages
Bile duct blockages usually occur when primary sclerosing cholangitis progresses. However, narrowed or blocked bile ducts may indicate cancer. Check below some treatments used to open clogged bile ducts:
- Balloon dilation – During this procedure, doctors can open blockages by using a slender tube with an inflatable balloon at its tip (also called a balloon catheter). When it reaches the narrowed or blocked part of the bile duct, it is inflated, which helps keep it open.
- Stent placement – It involves an endoscope and attached tools to place a small stent (plastic tube) in the clogged bile duct to hold it open.
Liver Transplant
Healthcare providers usually recommend this treatment for people with end-stage liver disease. However, this is the only treatment that may cure the primary sclerosing cholangitis. Unfortunately, this disease may recur in some people even after a liver transplant.
Home Remedies
People who are diagnosed with primary sclerosing cholangitis may consider the following tips to care for their liver. Examples include:
- Do not drink alcoholic beverages
- Get vaccinated against hepatitis A and B
- Use chemicals carefully at home and work
- Exercise regularly to maintain a healthy weight
- Administer medicines exactly as prescribed by your doctor
Frequently Asked Questions
What is the life expectancy of someone with primary sclerosing cholangitis?
Generally, the survival rate of people with this disease ranges between 9.3 to 18 years. This survival rate is based on people without a liver transplant. Moreover, a recent study in the Netherlands noticed that the median transplant-free survival rate is about 21.3 years.
Can primary sclerosing cholangitis be cured?
Yes, the only way to cure the condition is a liver transplant. However, some people may notice that the disease returns even after the transplant. Medicines that can be used to slow or reverse liver damage have not been found.
What is the most common cause of sclerosing cholangitis?
While experts cannot identify the exact cause of the disease, they believe that a combination of factors causes the condition. These include:
- Immune system disorders
- Abnormal genes
- Gut flora and microbiome changes
- Bile duct injuries caused by bile acid
Ask your healthcare provider if you have additional questions.
