Primary biliary cholangitis is a type of liver disease that damages the bile ducts. Early treatment may help prevent liver failure.
Primary biliary cholangitis, previously called primary biliary cirrhosis, is a chronic disease in which the bile ducts in your liver are slowly destroyed.
Bile is a fluid made in your liver. It aids with digestion and helps you absorb certain vitamins. It also helps your body get rid of cholesterol, toxins and worn-out red blood cells. Chronic inflammation in the liver can lead to bile duct damage, irreversible scarring of liver tissue (cirrhosis) and eventually, 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 medication can slow liver damage, especially if treatment begins early.
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.
More than half the 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:
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 (T lymphocytes) start to collect in the liver. Normally, 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 lining 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 (fibrosis) that can lead to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to work properly.
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:
As liver damage worsens, primary biliary cholangitis can cause serious health problems, including:
Your doctor 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.
Imaging tests may not be needed. However, they may help your doctor 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 doctor may perform a liver biopsy. A small sample of liver tissue is removed through a small incision using a thin needle. It's examined in a laboratory, either to confirm the diagnosis or to determine the extent (stage) of the disease.
There's no cure for primary biliary cholangitis, but medications are available to help slow the progression of the disease and prevent complications. Options include:
Your doctor may recommend treatments to control the signs and symptoms of primary biliary cholangitis and make you more comfortable.
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.
Artificial tears and saliva substitutes, available over-the-counter or by prescription, can help ease dry eyes and mouth. Chewing gum or sucking on hard candy also can help you make more saliva and relieve dry mouth.
Certain complications are commonly associated with primary biliary cholangitis. Your doctor may recommend:
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:
Living with a chronic liver disease with no cure can be frustrating. Fatigue alone can have a profound impact on your quality of life. Each person finds ways to cope with the stress of a chronic disease. In time, you'll find what works for you. Here are some ways to get started:
If you have signs or symptoms that worry you, make an appointment with your doctor. If you're diagnosed with primary biliary cholangitis, you may be referred to a doctor who specializes in disorders of the digestive system (gastroenterologist) or liver diseases (hepatologist).
Because there's often a lot to cover during your appointment, it's a good idea to arrive well prepared.
Preparing a list of questions can help you make the most of your time with your doctor. For primary biliary cholangitis, some basic questions to ask your doctor include:
In addition to the questions that you've prepared to ask your doctor, don't hesitate to ask other questions that come to mind during your appointment.
Your doctor is likely to ask you a number of questions. Being ready to answer them may give you more time to discuss a concern in greater detail. Your doctor may ask: