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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.

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:

• Fatigue.
• Itchy skin.

Later signs and symptoms may include:

• Yellowing of the skin and eyes, called jaundice.
• Dry eyes and mouth.
• Pain in the upper right abdomen.
• Swelling of the spleen, called splenomegaly.
• Bone, muscle or joint pain.
• Swollen feet and ankles.
• Buildup of fluid in the abdomen due to liver failure, called ascites.
• Fatty deposits, called xanthomas, on the skin around the eyes, eyelids or in the creases of the palms, soles, elbows or knees.
• Darkening of the skin that's not related to sun exposure, called hyperpigmentation.
• Weak and brittle bones, called osteoporosis, which can lead to fractures.
• High cholesterol.
• Diarrhea that may include greasy stools, called steatorrhea.
• Underactive thyroid, called hypothyroidism.
• Weight loss.

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:

• Sex. Most people with primary biliary cholangitis are women.
• Age. It's most likely to occur in people 30 to 60 years old.
• Genetics. You're more likely to get the condition if you have a family member who has or had it.
• Geography. It's most common in people of northern European descent, but primary biliary cholangitis can affect all ethnicities and races.

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

• Infections, such as a urinary tract infection.
• Smoking cigarettes, especially over long periods of time.
• Exposure to toxic chemicals, such as in certain work environments.

Complications

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

• Liver scarring, called cirrhosis. Cirrhosis makes it difficult for your liver to work and may lead to liver failure. It means the later stage of primary biliary cholangitis. People with primary biliary cholangitis and cirrhosis have a poor medical outlook. They also have a higher risk of other complications.
• Increased pressure in the portal vein, called portal hypertension. Blood from your intestine, spleen and pancreas enters your liver through a large blood vessel called the portal vein. When scar tissue from cirrhosis blocks normal blood flow through your liver, blood backs up. This causes increased pressure inside the vein. Also, because blood doesn't flow correctly through your liver, drugs and other toxins aren't filtered properly from your bloodstream.
• Enlarged veins, called varices. When blood flow through the portal vein is slowed or blocked, blood may back up into other veins. It usually backs up into those in your stomach and esophagus. Increased pressure may cause delicate veins to break open and bleed. Bleeding in the upper stomach or esophagus is a life-threatening emergency. It requires immediate medical care.
• Enlarged spleen, called splenomegaly. Your spleen may become swollen with white blood cells and platelets. This is because your body no longer filters toxins out of the bloodstream as it should.
• Gallstones and bile duct stones. If bile cannot flow through the bile ducts, it may harden into stones in the ducts. These stones can cause pain and infection.
• Liver cancer. Liver scarring increases your risk of liver cancer. If you have liver scarring, you'll need regular cancer screening.
• Weak bones, called osteoporosis. People with primary biliary cholangitis have an increased risk of weak, brittle bones that may break more easily.
• Vitamin deficiencies. Not having enough bile affects your digestive system's ability to absorb fats and the fat-soluble vitamins, A, D, E and K. Because of this, some people with advanced primary biliary cholangitis may have low levels of these vitamins. Low levels can result in a variety of health problems, including night blindness and bleeding disorders.
• High cholesterol. Up to 80% of people with primary biliary cholangitis have high cholesterol.
• Decreased mental function, called hepatic encephalopathy. Some people with advanced primary biliary cholangitis and cirrhosis have personality changes. They also may have problems with memory and concentration.
• Increased risk of other disease. Primary biliary cholangitis is associated with other disorders, including those that affect the thyroid, skin and joints. It also can be associated with dry eyes and mouth, a disorder called Sjogren's syndrome.

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:

• Liver tests. These blood tests check the levels of certain proteins that may signal liver disease and bile duct injury.
• Antibody tests for signs of autoimmune disease. Blood tests may be done to check for anti-mitochondrial antibodies, also known as AMAs. These substances almost never occur in people without the disease, even if they have other liver disorders. Therefore, a positive AMA test is considered a very reliable sign of the disease. However, a small number of people with primary biliary cirrhosis don't have AMAs.
• Cholesterol test. More than half the people with primary biliary cholangitis have extreme increases in blood fats, including total cholesterol level.

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:

• Ultrasound. Ultrasound uses high-frequency sound waves to produce images of structures inside your body.
• FibroScan. Using an ultrasound-like probe, this test can detect scarring of the liver.
• Magnetic resonance cholangiopancreatography, also known as MRCP. This special MRI creates detailed images of your organs and bile ducts.
• Magnetic resonance elastography, also known as MRE. MRI is combined with sound waves to create a visual map of internal organs, called an elastogram. The test is used to detect hardening of your liver that might be a sign of cirrhosis.

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:

• Ursodeoxycholic acid. This medicine, also known as UDCA or ursodiol (Actigall, Urso), is commonly used first. It helps move bile through your liver. UDCA doesn't cure primary biliary cholangitis, but it seems to improve liver function and reduce liver scarring. It's less likely to help with itching and fatigue. Side effects may include weight gain, hair loss and diarrhea.
• Obeticholic acid (Ocaliva). Studies show that when obeticholic acid is given alone or combined with ursodiol for 12 months, it can help improve liver function and slow liver fibrosis. However, its use is often limited because it can cause increased itching.
• Fibrates (Tricor). Researchers aren't exactly sure how this medicine works to help ease primary biliary cholangitis symptoms. But, when taken with UDCA, it has reduced liver inflammation and itching in some people. More studies are needed to determine long-term benefits.
• Budesonide. When combined with UDCA, the corticosteroid budesonide may be of potential benefit for primary biliary cholangitis. However, this medicine is associated with steroid-related side effects for people with more advanced disease. More long-term trials are necessary before budesonide can be recommended for treating this condition.
• Liver transplant. When medicines no longer control primary biliary cholangitis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant replaces your diseased liver with a healthy one from a donor. Liver transplantation is associated with very good long-term outcomes for people with primary biliary cholangitis. However, sometimes the disease comes back several years later in the transplanted liver.

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

• Antihistamines are commonly used to reduce itching. They may help with sleep if itching keeps you awake. Antihistamines may include diphenhydramine, hydroxyzine hydrochloride and loratadine.
• Cholestyramine is a powder that may stop itching. It must be mixed with food or liquids.
• Rifampin is an antibiotic that may stop itching. Exactly how it does this is unknown. Researchers think it may block the brain's response to itch-inducing chemicals in the blood.
• Opioid antagonists such as those containing naloxone and naltrexone may help itching related to liver disease. Like rifampin, these medicines seem to reduce the itching sensation by acting on your brain.
• Sertraline is a medicine that increases serotonin in the brain, called a selective serotonin reuptake inhibitor, or SSRI. It can help reduce 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:

• Vitamin and mineral supplements. If your body isn't absorbing vitamins or other nutrients, you may need to take vitamins A, D, E and K. You also may need calcium, folic acid or iron supplements.
• Medicine to lower cholesterol. If you have high cholesterol levels in your blood, your healthcare team may recommend taking a medicine known as a statin to help lower your levels.
• Medicines to treat bone loss. If you have weak or thinning bones, called osteoporosis, you may be prescribed medicines or supplements, such as calcium and vitamin D, to reduce bone loss and improve bone density. Exercise such as walking and using light weights most days of the week can help increase your bone density.
• Treatment for increased pressure in the portal vein, called portal hypertension. Your healthcare team is likely to screen and monitor you for portal hypertension and enlarged veins if you have more advanced scarring from liver disease. Fluid in your abdomen is a common side effect of portal hypertension. For mild fluid in the abdomen, your healthcare team may only recommend limiting salt in your diet. More-severe cases may require medicines known as diuretics or a procedure to drain the fluid called paracentesis.

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:

• Choose reduced-sodium foods. Sodium adds to tissue swelling and to the buildup of fluid in your abdomen. Look for low-sodium foods or naturally sodium-free foods.
• Avoid eating oysters or other raw shellfish. Such seafood can carry infection-causing bacteria. Infections can be dangerous for people with liver disease.
• Exercise most days of the week. Exercise may reduce your risk of bone loss.
• Don't smoke. If you don't smoke, don't start. If you currently smoke, talk with a healthcare professional about strategies to help you quit.
• Avoid alcohol. Your liver processes the alcohol you drink. The added stress can cause liver damage. Generally, people with primary biliary cholangitis should not drink alcohol.
• Check with your healthcare team before starting new medicines or dietary supplements. Because your liver isn't working normally, you'll likely be more sensitive to the effects of medicines and some dietary supplements. Check with your healthcare team before taking anything new.

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:

• Learn about your condition. The more you understand about primary biliary cholangitis, the more active you can be in your own care. In addition to talking with your healthcare team, look for information at your local library and on websites affiliated with reputable organizations such as the American Liver Foundation.
• Take time for yourself. Eating well, exercising and getting enough rest can help you feel better. Try to plan ahead for times when you may need more rest.
• Get help. If friends or family want to help, let them. Primary biliary cholangitis can be exhausting, so accept the help if someone wants to do your grocery shopping, wash a load of laundry or cook your dinner. Tell those who offer to help what you need.
• Seek support. Strong relationships can help you maintain a positive attitude. If friends or family have a hard time understanding your illness, you may find that a support group can be helpful.

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

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may not seem related to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medicines, vitamins and supplements that you're taking and the doses.
• Ask a family member or friend to come with you. Sometimes it can be difficult to remember all of the information provided to you during an appointment. Someone who accompanies you may remember something that you missed or forgot.
• Write down questions to ask your healthcare team.

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:

• What's the most likely cause of my symptoms?
• What kinds of tests do I need to confirm the diagnosis? Do these tests require any special preparation?
• How severe is the damage to my liver?
• What treatments do you recommend for me?
• Will I need a liver transplant?
• What types of side effects can I expect from treatment?
• Are there any other treatment options?
• Do I need to change my diet?
• Are there brochures or other printed material that I can take with me? What websites do you recommend?

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:

• What symptoms have you been experiencing?
• When did you first notice them?
• Do you always have symptoms, or do they come and go?
• How severe are your symptoms?
• What, if anything, makes your symptoms better or worse?
• Has anyone in your family ever been diagnosed with primary biliary cholangitis?
• Do you have any ongoing health conditions?
• Do you have a history of hepatitis or other liver disease?
• Does anyone in your family have liver disease?
• How much alcohol do you drink?
• What medicines are you taking?
• Do you take any herbal or natural remedies?