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.

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.

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

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
   

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.

Sources:

https://www.mayoclinic.org/diseases-conditions/primary-biliary-cholangitis-pbc/symptoms-causes/syc-20376874

Our partner Intercept run two webinars for health care professionals  in July this year, dedicated to following topics:

• Optimizing PBC care in the new environment: What has the pandemic taught us?
• New evidence that shapes PBC clinical practice in 2020

If you are a health care professional, please sign-in at the right upper corner with your DocCheck account to watch these videos in English.