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Primary biliary cholangitis

Medically reviewed by Drugs.com. Last updated on Nov 14, 2023.

Overview

Primary biliary cholangitis is an autoimmune disease in which the bile ducts are inflamed and slowly destroyed. It previously was called primary biliary cirrhosis.

Bile is a fluid made in the liver. It helps with digestion and absorbing certain vitamins. It also helps the body absorb fats and get rid of cholesterol, toxins and worn-out red blood cells. Ongoing inflammation in the liver can lead to bile duct inflammation and damage known as cholangitis. At times, this can lead to permanent scarring of liver tissue, called cirrhosis. It also can eventually lead to liver failure.

Although it affects both sexes, primary biliary cholangitis mostly affects women. It's considered an autoimmune disease, which means your body's immune system is mistakenly attacking healthy cells and tissue. Researchers think a combination of genetic and environmental factors triggers the disease. It usually develops slowly. At this time, there's no cure for primary biliary cholangitis, but medicines may slow liver damage, especially if treatment begins early.

Bile duct damage

The bile ducts carry bile from your liver to your small intestine. When bile ducts become damaged, bile can back up into the liver, causing damage to liver cells. This damage can lead to liver failure.

Symptoms

More than half of people with primary biliary cholangitis do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing. Symptoms eventually develop over the next 5 to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes.

Common early symptoms include:

Later signs and symptoms may include:

Causes

It's not clear what causes primary biliary cholangitis. Many experts consider it an autoimmune disease in which the body turns against its own cells. Researchers believe this autoimmune response may be triggered by environmental and genetic factors.

The liver inflammation seen in primary biliary cholangitis starts when certain types of white blood cells called T cells, also known as T lymphocytes, start to collect in the liver. Usually, these immune cells detect and help defend against germs, such as bacteria and viruses. But in primary biliary cholangitis, they mistakenly destroy the healthy cells that line the small bile ducts in the liver.

Inflammation in the smallest ducts spreads and eventually damages other cells in the liver. As the cells die, they're replaced by scar tissue, also known as fibrosis, that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.

Risk factors

The following factors may increase your risk of primary biliary cholangitis:

Researchers think that genetic factors combined with certain environmental factors trigger primary biliary cholangitis. These environmental factors may include:

Complications

As liver damage worsens, primary biliary cholangitis can cause serious health problems, including:

Diagnosis

Your healthcare professional will ask you about your health history and your family's health history, and perform a physical exam. The following tests and procedures may be used to diagnose primary biliary cholangitis.

Blood tests:

Imaging tests may help your healthcare team confirm a diagnosis or rule out other conditions with similar signs and symptoms. Imaging tests looking at the liver and bile ducts may include:

If the diagnosis is still uncertain, your healthcare professional may perform a liver biopsy. A small sample of liver tissue is removed through an incision using a thin needle. It's then tested in a lab, either to confirm the diagnosis or to determine the extent of the disease.

Treatment

Treating the disease

There's no cure for primary biliary cholangitis, but medicines are available to help slow the progression of the disease and prevent complications. Options include:

Treating the symptoms

Your healthcare team may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable.

Treatment for fatigue

Primary biliary cholangitis causes fatigue. But your daily habits, proper diet and exercise, and other health conditions can affect how tired you feel. It is important to also be tested to exclude thyroid disease since it is more common in people with primary biliary cholangitis.

Treatment for itching

Treatment for dry eyes and mouth

Artificial tears and saliva substitutes can help ease dry eyes and mouth. They may be available with or without a prescription. Chewing gum or sucking on hard candy also can help you make more saliva and relieve dry mouth.

Treating the complications

Certain complications are commonly associated with primary biliary cholangitis. Your healthcare team may recommend:

Lifestyle and home remedies

You may feel better if you take good care of your overall health. Here are some things you can do to improve some primary biliary cholangitis symptoms and, possibly, help prevent certain complications:

Coping and support

Living with an ongoing liver disease with no cure can be frustrating. Fatigue alone can have a large impact on your quality of life. Each person finds ways to cope with the stress of an ongoing disease. In time, you'll find what works for you. Here are some ways to get started:

Preparing for an appointment

Make an appointment with a doctor or other healthcare professional if you have any symptoms that worry you.

If your healthcare professional thinks you might have primary biliary cholangitis, you may be referred to a doctor who specializes in disorders of the digestive system, called a gastroenterologist. You also may be referred to a doctor who specializes in liver diseases, called a hepatologist.

Because appointments can be brief, it's a good idea to be prepared. Here's some information to help you get ready.

What you can do

Your time with your healthcare team is limited, so preparing a list of questions can help you make the most of your time together. List your questions from most important to least important in case time runs out. For primary biliary cholangitis, some basic questions to ask include:

Don't hesitate to ask other questions.

What to expect from your doctor

You are likely to be asked a number of questions. Being ready to answer them may give you more time to further discuss a concern. You may be asked:

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