Primary Biliary Cholangitis

Your liver makes a fluid called bile, which helps your body digest food and absorb nutrients, like vitamins and fats. Bile also helps remove waste products like cholesterol and old blood cells. Bile travels through tubes called bile ducts to your small intestine.

Sometimes, these bile ducts get inflamed and damaged. This can happen in a condition called primary biliary cholangitis (PBC). Previously, this was called primary biliary cirrhosis. In PBC, the body's immune system, which is supposed to fight off infections, mistakenly attacks and damages the bile ducts. This inflammation can lead to the bile backing up into the liver, causing more damage to the liver cells.

PBC is most common in women. Scientists believe that a mix of inherited traits (genes) and things in the environment play a role in causing the disease. PBC usually develops gradually over time.

Unfortunately, there's no cure for PBC, but medications can help slow down the damage to the liver, especially if treatment starts early. The ongoing inflammation can cause permanent scarring in the liver (cirrhosis), which can eventually lead to liver failure.

Many people with primary biliary cholangitis (PBC) don't show any clear signs of the disease when it's first found. Sometimes, doctors discover it during routine blood tests that are done for other reasons. In other cases, symptoms might not appear for 5 to 20 years after the disease begins. Unfortunately, people who do have symptoms when diagnosed often have worse health outcomes in the long run.

Early warning signs can include tiredness and an itchy skin.

As the disease progresses, other symptoms might develop, including:

• Jaundice: This is a yellowing of the skin and eyes.
• Dry eyes and mouth: These are common signs of dehydration and can also indicate other health issues.
• Abdominal pain: This is often felt in the upper right side of the belly.
• Swollen spleen (splenomegaly): The spleen is an organ that helps filter blood. When it swells, it can cause discomfort.
• Bone, muscle, or joint pain: This pain can be a symptom of various conditions, so it's important to get it checked.
• Swollen feet and ankles: This can occur due to fluid buildup.
• Ascites: This is a buildup of fluid in the abdomen, often a sign of advanced liver problems.
• Xanthomas: These are fatty deposits that can appear on the skin, often around the eyes, eyelids, or in creases like palms, soles, elbows, and knees.
• Hyperpigmentation: This is darkening of the skin that isn't caused by sun exposure.
• Osteoporosis: This is a condition that weakens bones, making them more prone to fractures.
• High cholesterol: High cholesterol is associated with various health issues.
• Greasy stools (steatorrhea): This is a type of diarrhea that includes fatty stools.
• Underactive thyroid (hypothyroidism): The thyroid is a gland that regulates many bodily functions. An underactive thyroid can lead to a variety of symptoms.
• Weight loss: This can be a symptom of many different conditions, so it's essential to talk to a doctor.

It's crucial to remember that this list isn't exhaustive, and not everyone with PBC will experience all these symptoms. If you have concerns about any of these symptoms, it's best to see a doctor for proper diagnosis and treatment.

Primary biliary cholangitis (PBC) is a condition where the liver gets inflamed. Scientists aren't completely sure what causes it, but many believe it's an autoimmune disease. This means the body's immune system, which is normally designed to fight off infections like bacteria and viruses, mistakenly attacks healthy cells in the liver.

The problem begins when a type of immune cell called a T cell (or T lymphocyte) builds up in the liver. Normally, T cells are vital for fighting off illness. But in PBC, these T cells attack and damage the cells lining the tiny tubes (ducts) that carry bile out of the liver.

This attack on the bile ducts causes inflammation that spreads throughout the liver. As the liver cells are damaged and die, they are replaced by scar tissue, a process called fibrosis. Over time, this scarring can lead to cirrhosis. Cirrhosis is like a permanent, damaging scar on the liver, making it hard for the liver to perform its important jobs, like filtering blood and processing nutrients.

Primary biliary cholangitis (PBC) is a condition that affects the bile ducts in the liver. Several things can increase your chances of getting PBC.

Who is at risk?

• Gender: Women are much more likely to develop PBC than men.
• Age: Most people diagnosed with PBC are between the ages of 30 and 60.
• Family history: If someone in your family has PBC, you have a higher chance of developing it yourself.
• Ethnicity: While PBC is more common in people of northern European descent, it can affect people of all backgrounds and ethnicities.

What might cause PBC?

Scientists believe that PBC happens when a person's genes interact with certain environmental factors. These environmental triggers could include:

• Infections: Having an infection, such as a urinary tract infection (UTI), might play a role. It's important to note that infections alone don't cause PBC, but they might be a piece of the puzzle.
• Smoking: Long-term cigarette smoking is linked to a higher risk of PBC. The more you smoke, and the longer you smoke, the greater the risk.
• Exposure to toxins: Exposure to certain chemicals at work or in the environment may also increase the risk of PBC. This isn't about a single exposure, but rather long-term or repeated contact with these substances.

It's important to remember that these factors don't guarantee that you'll get PBC. They simply increase your chances. If you have concerns about your risk, it's always best to talk to a doctor.

As primary biliary cholangitis (PBC) damages the liver, it can lead to serious health problems. These complications worsen as the liver damage progresses.

Liver Scarring (Cirrhosis): One major complication is cirrhosis, where scar tissue builds up in the liver. This makes the liver less able to function properly, potentially leading to liver failure. Cirrhosis is a late-stage complication of PBC, and unfortunately, people with both PBC and cirrhosis often have a less favorable prognosis (outlook) and a higher chance of developing other problems.

Spleen Enlargement (Splenomegaly): The spleen, part of the immune system, can become swollen (enlarged). This happens because the liver's inability to filter toxins from the blood properly, puts a strain on the spleen. The enlarged spleen is usually a sign of more advanced liver disease.

Gallstones and Bile Duct Stones: Bile, a fluid produced by the liver, helps digest food. If bile flow is blocked in the tubes (bile ducts), it can harden and form stones. These stones can cause pain and infection.

Liver Cancer: Liver scarring increases the risk of developing liver cancer. Regular checkups for liver cancer are important for people with PBC and cirrhosis.

Weak Bones (Osteoporosis): People with PBC have a higher chance of developing osteoporosis, a condition where bones become weak and brittle, making them more prone to fractures.

Vitamin Deficiencies: The liver makes bile, which helps your body absorb important vitamins like A, D, E, and K. If the liver isn't working well in PBC, your body might not absorb enough of these vitamins. Low levels of these vitamins can lead to various health problems, including vision problems (night blindness) and bleeding issues.

High Cholesterol: A significant number of people with PBC (around 80%) experience high cholesterol levels.

Mental Function Impairment (Hepatic Encephalopathy): In advanced cases of PBC, particularly when combined with cirrhosis, some individuals may experience changes in their personality and problems with memory and concentration. This is due to toxins building up in the blood that affect the brain.

Increased Risk of Other Diseases: PBC is often linked to other health conditions, such as problems with the thyroid, skin, and joints. It can also be associated with a condition called Sjogren's syndrome, which involves dry eyes and mouth.

Diagnosing Primary Biliary Cholangitis (PBC)

Doctors use several methods to diagnose PBC, a condition affecting the bile ducts in the liver. They first take a detailed medical history, including your family's health history, and perform a physical exam. Various tests and procedures help confirm the diagnosis.

Blood Tests:

• Liver Function Tests: These tests measure proteins in your blood. High levels of certain proteins can indicate liver damage or problems with the bile ducts.
• Antibody Tests: Blood tests look for specific antibodies, like anti-mitochondrial antibodies (AMAs). AMAs are almost always present in people with PBC, making a positive result a strong indicator of the disease. However, a small portion of people with PBC may not have AMAs.
• Cholesterol Test: Many people with PBC have unusually high levels of blood fats, including cholesterol.

Imaging Tests:

• Ultrasound: This uses sound waves to create images of the liver and bile ducts. It helps the doctor see the general structure of these organs.
• FibroScan: Similar to ultrasound, this test uses a probe to measure liver scarring (fibrosis).
• MRCP (Magnetic Resonance Cholangiopancreatography): This type of MRI scan creates detailed images of the bile ducts. This helps the doctor see if there are blockages or other problems.
• MRE (Magnetic Resonance Elastography): This advanced MRI technique combines sound waves with MRI to create a visual map of how stiff the liver is. This can help detect liver hardening, a sign of cirrhosis, a serious complication of PBC.

Liver Biopsy:

If the diagnosis is still uncertain after other tests, a doctor may perform a liver biopsy. This involves taking a small tissue sample from the liver using a needle. The sample is then examined in a lab to confirm the diagnosis and assess the severity of the disease.

Important Note: A team of experienced doctors at Mayo Clinic can provide comprehensive care for PBC. They can explain the results of your tests and discuss treatment options.

Primary Biliary Cholangitis: Treatment and Management

Primary biliary cholangitis (PBC) is a disease that affects the bile ducts in the liver. While there's no cure, treatments can help slow the disease's progress and manage its symptoms.

Medications to Slow Disease Progression:

Several medications can help manage PBC. The most common first choice is ursodeoxycholic acid (UDCA), sometimes called ursodiol. It helps bile flow through the liver, potentially improving liver function and reducing scarring. However, it might not be as effective in relieving itching or fatigue. Possible side effects include weight gain, hair loss, and diarrhea.

Obeticholic acid (Ocaliva) is another option. Studies show it can improve liver function and slow liver damage when used alone or with UDCA for a year. A downside is that it can sometimes increase itching.

Fibrates (like Tricor) are used with UDCA in some cases. While the exact way they help isn't fully understood, they may reduce liver inflammation and itching in some people. More research is needed to confirm long-term benefits.

Budesonide, a corticosteroid, is sometimes used with UDCA. It might be helpful, but it can cause side effects similar to other steroids, especially in people with advanced disease. More long-term studies are needed.

A liver transplant is an option if medications are no longer effective and liver function is failing. A transplant replaces the diseased liver with a healthy one from a donor. Transplants generally have very good long-term outcomes, although the PBC can sometimes return in the transplanted liver after several years.

Managing Symptoms:

PBC can cause various symptoms, and treatments focus on making you more comfortable.

• Fatigue: PBC causes fatigue, but other factors like diet, exercise, and underlying health conditions can also play a role. Thyroid problems are more common in people with PBC, so it's important to rule those out. Lifestyle changes, like regular exercise and a healthy diet, can help.

• Itching: Antihistamines (like diphenhydramine, hydroxyzine, or loratadine) are often used to reduce itching. These can also help with sleep if itching disrupts it. Cholestyramine (a powder) mixed with food or liquid may also help. Rifampin, an antibiotic, may also reduce itching, although its exact mechanism isn't completely understood. Other medications, such as opioid antagonists (containing naloxone or naltrexone) and sertraline (an SSRI), may also help by affecting the brain's response to itching.

• Dry Eyes and Mouth: Artificial tears and saliva substitutes can ease dry eyes and mouth. Chewing gum or sucking on hard candies can also stimulate saliva production.

Managing Complications:

PBC can lead to various complications:

• Vitamin and Mineral Deficiencies: If your body isn't absorbing vitamins (A, D, E, K) or minerals (calcium, folic acid, iron), supplements may be necessary.

• High Cholesterol: Statins may be prescribed to lower high cholesterol levels.

• Bone Loss (Osteoporosis): If you have weak bones, calcium, vitamin D supplements, and exercises like walking and light weight training can help maintain bone density.

• Portal Hypertension: If you have advanced liver scarring, your doctor will monitor for portal hypertension (increased pressure in the portal vein). Mild cases may only require a low-salt diet. More severe cases may need diuretics or a procedure called paracentesis to drain fluid buildup in the abdomen.

Important Note: This information is for general knowledge and does not constitute medical advice. Always consult with your healthcare provider for personalized guidance and treatment plans.

Living with a chronic liver condition can be tough, especially since there's no cure. Feeling tired all the time can really affect how you live your life. Everyone handles the stress of a long-term illness differently. Over time, you'll figure out what works best for you. Here are some things that can help you get started:

Learn about your condition: Understanding your specific liver disease (like primary biliary cholangitis) is key to managing it. The more you know, the more involved you can be in your own treatment. Talk to your doctor, nurse, or other healthcare providers. Also, look for reliable information at your local library, and on websites from organizations like the American Liver Foundation. These resources can give you a better understanding of your situation and what to expect.

Take care of yourself: Taking good care of your body can significantly improve your well-being. Eating healthy foods, getting regular exercise, and getting enough sleep are crucial. Try to schedule extra rest periods when you need them, so you don't burn out.

Ask for help: Don't be afraid to accept help from friends and family. Managing a chronic illness can be tiring, so if someone offers to help with errands like grocery shopping, laundry, or cooking, say yes! Let them know exactly what you need help with. For example, "I'd really appreciate it if you could pick up groceries this week."

Find support: Strong relationships are important for maintaining a positive outlook. If friends or family struggle to understand what you're going through, a support group can be invaluable. Connecting with others who have similar experiences can provide comfort, understanding, and practical advice. Sharing your experiences and learning from others can make a big difference.