Primary Biliary Cholangitis

Primary biliary cholangitis is a long-term liver condition where the immune system mistakenly attacks the small tubes in the liver that carry bile, leading to gradual damage that can worsen over time without treatment.

Table of contents

• What is Primary Biliary Cholangitis?
• Who Gets Primary Biliary Cholangitis?
• Symptoms of Primary Biliary Cholangitis
• Causes and Risk Factors
• How is Primary Biliary Cholangitis Diagnosed?
• Treatment Options
• Complications and Related Conditions
• Living with Primary Biliary Cholangitis

What is Primary Biliary Cholangitis?

Primary biliary cholangitis, often called PBC, is a chronic disease (long-lasting condition) that affects the liver^[1]. The condition used to be called primary biliary cirrhosis, but the name was changed because most people with PBC do not have cirrhosis (severe scarring of the liver) when they are diagnosed^[1][6].

In PBC, the small tubes inside the liver called bile ducts become inflamed and are eventually destroyed^[2]. Bile is a fluid made by the liver that helps digest food, especially fats, and removes waste products from the body^[1]. These bile ducts normally carry bile from the liver through the gallbladder to the small intestine. When the ducts are damaged, bile cannot flow properly. It backs up inside the liver, causing damage to liver tissue^[1][2].

Over time, this ongoing damage can lead to permanent scarring of the liver, which is called cirrhosis. Eventually, if left untreated, PBC can cause liver failure^[1][2]. However, PBC develops slowly, often over many years, and not everyone will reach this advanced stage^[2].

• Liver
• Bile ducts
• Gallbladder

Who Gets Primary Biliary Cholangitis?

PBC primarily affects women much more than men, with about 9 out of 10 people diagnosed being female^[2][5]. In the United States, researchers estimate that about 58 out of every 100,000 women and about 15 out of every 100,000 men have PBC^[5].

Most people are diagnosed after age 40, with the average age at diagnosis being around 60 years old^[2][5]. The condition is more common in certain parts of the world, including Scotland, Scandinavia, and Northeast England^[2].

People who have a parent or sibling with PBC, particularly an identical twin, are more likely to develop the condition themselves. This suggests that genetics may play a role^[2][5]. The condition is also more common in people who have a personal or family history of other autoimmune diseases (conditions where the immune system attacks the body’s own tissues)^[2].

Symptoms of Primary Biliary Cholangitis

More than half of people with PBC do not have any noticeable symptoms when they are first diagnosed^[1]. Many people find out they have the disease when blood tests are done for other reasons, such as routine check-ups^[1][6].

When symptoms do appear, they usually develop slowly over time. The most common early symptoms are^[2][9]:

• Fatigue (extreme tiredness) affecting about 65% of people with PBC
• Itchy skin (also called pruritus) affecting about 55% of people

These symptoms can affect different people in different ways. Some people may have mild symptoms while others experience severe discomfort. Importantly, how strong your symptoms are does not always match how advanced the disease is^[2].

Other symptoms that may occur include^[6][9]:

• Dry eyes and mouth
• Bone and joint aches
• Pain or discomfort in the upper right side of the abdomen (belly)

As the disease progresses and liver damage becomes more severe, additional symptoms may develop, such as yellowing of the skin and eyes (called jaundice) and swelling in the abdomen^[2][6].

Causes and Risk Factors

PBC is believed to be an autoimmune disease, which means the body’s immune system mistakenly attacks healthy cells in the bile ducts^[1][5]. Normally, the immune system protects the body from infections and illness. In PBC, however, it becomes overactive and attacks the bile duct cells as if they were harmful invaders.

The exact reason why this happens is not clearly understood. Researchers believe that PBC develops through a combination of genetic factors and environmental triggers^[7]. This means that some people may be born with genes that make them more likely to develop PBC, but something in their environment may trigger the disease to start.

Possible environmental factors that may trigger PBC include^[7]:

• Urinary tract infections
• Use of reproductive hormone replacement
• Exposure to certain chemicals like nail polish
• Cigarette smoking

People with other autoimmune conditions are at higher risk of developing PBC. These conditions include celiac disease, systemic sclerosis, thyroid disease, and Sjögren syndrome^[5][7].

How is Primary Biliary Cholangitis Diagnosed?

To diagnose PBC, your doctor will start by asking about your health history and your family’s health history, and will perform a physical exam^[11]. Several tests may be used to confirm the diagnosis.

Blood tests are the main way PBC is diagnosed^[6][11]. These tests check:

• Levels of certain liver proteins that may signal liver disease and bile duct injury
• The presence of anti-mitochondrial antibodies (AMAs), which are substances found in more than 90% of people with PBC^[7]. These antibodies almost never occur in people without the disease, so finding them is a very reliable sign of PBC
• Other antibodies called antinuclear antibodies (ANAs), which are often present in PBC
• Cholesterol levels, which can be extremely high in people with PBC^[11]

Imaging tests may be done to look at the liver and bile ducts. These can help rule out other problems with similar symptoms. Common imaging tests include^[11]:

• Ultrasound, which uses sound waves to create pictures of the liver
• FibroScan, a special ultrasound that measures liver scarring
• MRI (magnetic resonance imaging), which creates detailed images of the liver and bile ducts
• Magnetic resonance elastography (MRE), which detects hardening of the liver that might be a sign of cirrhosis

A liver biopsy may occasionally be recommended if the diagnosis is still uncertain^[7][11]. This involves safely removing a small sample of liver tissue to be studied under a microscope. The biopsy can help doctors assess liver damage and decide on the best treatment.

Treatment Options

While there is no cure for PBC, treatments are available to slow down the liver damage and help manage symptoms^[1][6]. Starting treatment early is important because it can help prevent the disease from getting worse.

Medications to Slow Disease Progression

Ursodeoxycholic acid (UDCA) is the main treatment for PBC^[13][14]. Although UDCA does not cure PBC, it can help slow down liver damage. People who respond well to UDCA early in the disease can live longer without needing a liver transplant^[14]. Once you start taking UDCA, you will likely need to take it for the rest of your life^[13].

If UDCA is not working well enough on its own, your doctor may prescribe additional medications^[13][14]:

• Obeticholic acid (OCA) works by improving bile flow and reducing inflammation. It may be used in combination with UDCA or alone if you cannot take UDCA. A possible side effect of OCA is itching^[13]
• Elafibranor reduces the amount of bile acid produced and makes it less damaging to the liver. Like OCA, it may be used with UDCA or alone^[13]

Treating Itchiness

Itchiness is one of the most frustrating symptoms of PBC. Colestyramine (also called cholestyramine) is a medicine widely used to treat itching associated with PBC^[13]. It comes in powder form that can be dissolved in water or fruit juice. You may need to take it for a few days before your symptoms improve.

If you are taking both UDCA and colestyramine, take them at different times. You should take UDCA at least 1 hour before colestyramine, or 4 to 6 hours afterwards^[13].

If colestyramine does not help your itchiness, your doctor may prescribe alternative medicines such as rifampicin (an antibiotic) or naltrexone^[13]. Using moisturizers regularly to prevent dry skin may also help reduce itching^[13].

Managing Fatigue

Unfortunately, no medicines are currently available to specifically treat fatigue associated with PBC^[13]. Your doctor may want to check for other possible causes of tiredness, such as low iron levels in the blood, sleep problems, or depression, which can be treated^[13].

Exercise can help improve fatigue from PBC, even though it may be hard to get moving when you feel tired^[13][18]. Many people notice that activity boosts their energy levels and improves sleep quality.

Liver Transplant

In rare cases where PBC leads to severe liver damage or liver failure, a liver transplant may be needed^[1][6]. A liver transplant is a major surgery where a damaged liver is replaced with a healthy liver from a donor.

Complications and Related Conditions

PBC can lead to several complications, especially if it reaches an advanced stage^[5][6]:

• High blood cholesterol levels, which may require treatment with medications called statins and lifestyle changes^[14]
• Osteoporosis, a condition where bones become weak and brittle. This is one of the most common complications of PBC^[5][6][18]. Your doctor may prescribe medicines to slow bone loss and improve bone density, and recommend supplements of calcium and vitamin D^[14]
• Vitamin deficiencies, particularly of fat-soluble vitamins A, D, E, and K. Your doctor may recommend taking vitamin supplements^[5][14]
• Portal hypertension, which is increased blood pressure inside the blood vessels in the abdomen^[6]
• Ascites, a build-up of fluid in the abdomen^[6]
• A slightly increased risk of developing liver cancer^[6]

People with PBC may also have other autoimmune diseases, including^[5]:

• Autoimmune thyroid diseases
• Raynaud’s disease
• Sjögren’s syndrome, which causes dry eyes and mouth
• Scleroderma
• Autoimmune hepatitis

Women with PBC may also have frequent urinary tract infections^[5].

Living with Primary Biliary Cholangitis

Living with PBC means making daily decisions that can help keep your liver and overall body healthier for longer^[18]. Here are some important steps you can take:

Follow a Healthy Diet

Following a Mediterranean-style diet typically works well for people with PBC^[18]. This means eating plenty of:

• Fruits and vegetables, which provide important vitamins
• Whole-grain foods like oatmeal, whole-grain bread, and brown rice
• Healthy fats like omega-3 fatty acids, which are easier for your liver to process

It is best to limit foods high in saturated fats, sugar, or sodium. Reducing salt in your diet may help prevent swelling^[17]. Alcohol should be minimized or avoided, as it can put extra stress on your liver^[18].

Staying hydrated by drinking water throughout the day is important for every part of the body and can help with symptoms like itchy skin, swelling, and fatigue^[17].

Exercise Regularly

Regular exercise is important for maintaining strong bones and can help guard against osteoporosis^[18]. Both aerobic exercise (like walking) and resistance training (strength exercises) are recommended. Exercise also helps fight fatigue by increasing blood flow and causing your body to release energy-building hormones^[18].

Prevent Falls

Because PBC can lead to weakened bones, preventing falls becomes important^[18]. You can make your home safer by:

• Clearing walkways of clutter and tripping hazards like shoes or cords
• Getting rid of throw rugs or securing them with tape
• Adding grab bars and secure mats to bathrooms and showers
• Lighting dark areas to eliminate shadows

Avoid Certain Medicines

The damage to your liver can affect its ability to process certain medicines^[6]. You may need to avoid medicines like aspirin and ibuprofen. Always tell any healthcare professional treating you that you have PBC, and ask your doctor about any medicines you should avoid.

Get Regular Check-Ups

It is important to regularly see a liver specialist to monitor the disease and look for any signs of progression^[18]. Different medications and treatments are available to address your specific needs. Your healthcare provider can also test for vitamin deficiencies and recommend appropriate supplements.

Stop Smoking

If you smoke, stopping is important. Smoking is a risk factor for developing PBC and can make liver disease worse^[6][19].

Take Care of Your Mental Health

Being diagnosed with a chronic disease can be challenging. It may take time to come to terms with having PBC, especially since it is an uncommon condition that most people have never heard of^[19]. Many people find it helpful to connect with others who have PBC through support groups, either in person or online. These groups provide a space to share experiences, frustrations, and advice with people who understand what you are going through.