Definition & Facts of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)

What is primary biliary cholangitis?

Primary biliary cholangitis is the new name for primary biliary cirrhosis, or PBC. Primary biliary cholangitis is a chronic disease in which the small bile ducts in the liver become injured and inflamed and are eventually destroyed. When there are no bile ducts, bile builds up and causes liver damage. This damage can lead to liver scarring, cirrhosis, and eventually liver failure. Primary biliary cholangitis is believed to be an autoimmune disease, which means that the immune system is overactive and attacks normal, healthy bile duct cells.

How common is primary biliary cholangitis?

Researchers estimate that in the United States, about 65 out of every 100,000 women and about 12 out of every 100,000 men have primary biliary cholangitis.¹

Who is more likely to get primary biliary cholangitis?

You are more likely to get primary biliary cholangitis if you

• are a woman
• are older than age 35
• have a parent or sibling—particularly an identical twin—with the disease
• are of Northern European descent

What other health problems do people with primary biliary cholangitis have?

People with primary biliary cholangitis may have certain autoimmune diseases, including

• autoimmune hepatitis
• autoimmune thyroid diseases—conditions in which the immune system attacks the thyroid gland
• celiac disease
• Raynaud’s disease
• Sjögren’s syndrome
• scleroderma

People with primary biliary cholangitis may also have frequent urinary tract infections.

What are the complications of primary biliary cholangitis?

Common complications of primary biliary cholangitis include

• high blood cholesterol levels
• osteoporosis
• fat-soluble vitamin deficiencies

These common complications can be prevented and treated.

Primary biliary cholangitis can also lead to cirrhosis and liver failure.

References