Primary Biliary Cholangitis

• With PBC, your body attacks tubes in your liver, which are called bile ducts, and causes bile to build up in the liver.
• Bile buildup can be toxic and can interfere with the liver’s ability to function.
• Pruritus (itching) and fatigue (feeling tired all over) are the most common symptoms of PBC; however, they are not related to how far the disease has progressed.

Cause

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.

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. 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.

Risk factors

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

• Sex. Most people with primary biliary cholangitis are women.
• Age. It's most likely to occur in people 30 to 60 years old.
• Genetic factors. You're more likely to get the condition if you have a family member who has or had it.

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

• Infections caused by bacteria, fungi or parasites
• Smoking
• Toxic chemicals

Symptoms

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. Symptoms eventually develop over the next five to 20 years. Those who do have symptoms at diagnosis typically have poorer outcomes.

Common early symptoms include:

• Fatigue
• Itchy skin
• Dry eyes and mouth

Later signs and symptoms may include:

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

Diagnosis

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.

Blood tests:

• Cholesterol test. More than half the people with primary biliary cholangitis have extreme increases in blood fats (lipids), including total cholesterol level.
• Liver tests. These blood tests check the levels of enzymes 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 (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.

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:

• Ultrasound. Ultrasound uses high-frequency sound waves to produce images of structures inside your body.
• Magnetic resonance cholangiopancreatography (MRCP). This special magnetic resonance imaging (MRI) exam creates detailed images of your organs and bile ducts.
• Magnetic resonance elastography (MRE). MRI is combined with sound waves to create a visual map (elastogram) of internal organs. The test is used to detect hardening of your liver that might be a sign of cirrhosis.
• Endoscopic retrograde cholangiopancreatography (ERCP). The doctor passes a thin, flexible tube down your throat and injects dye into the area of your small intestine where your bile ducts empty. A tiny camera attached to the end of the tube provides a picture of your bile ducts. This test may be done with or instead of an MRCP. But, it's invasive and may cause complications. With advances in MRI, it's usually not needed for diagnosis.

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.

Everyday life

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. Opt for low-sodium foods or naturally sodium-free foods, since sodium contributes to tissue swelling and to the buildup of fluid in your abdominal cavity (ascites).
• Never eat oysters or other raw shellfish. Such seafood can carry infection-causing bacteria, which can be dangerous for people with liver disease.
• Exercise most days of the week. Exercise may reduce your risk of bone loss.
• Avoid alcohol. Your liver processes the alcohol you drink, and the added stress can cause liver damage. Generally, people with primary biliary cholangitis should abstain from alcohol.
• Check with your doctor before starting new medications or dietary supplements. Because your liver isn't working normally, you'll likely be more sensitive to the effects of over-the-counter and prescription medications, as well as some dietary supplements, so check with your doctor before taking anything new.

Coping and support

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:

• 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 doctor, 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.