Primary Biliary Cholangitis
OVERVIEW
Primary Biliary Cholangitis (PBC) is a chronic autoimmune liver disease that affects the bile ducts within the liver. The bile ducts are responsible for carrying bile, a digestive fluid, from the liver to the small intestine.
In PBC, the immune system mistakenly attacks the bile ducts and causes inflammation and scarring. Over time, the scarring can block the flow of bile and cause damage to the liver. This can lead to a buildup of bile within the liver, which can cause liver cells to die and eventually lead to cirrhosis, a serious liver condition.
PBC is more common in women and typically develops in people over the age of 40. The symptoms of PBC can include fatigue, itching, yellowing of the skin and eyes (jaundice), joint pain, and digestive problems. In some cases, PBC may be asymptomatic for many years and may be discovered only after a routine blood test or liver function test.
Diagnosing PBC requires a combination of blood tests, liver function tests, and a liver biopsy. There is no cure for PBC, but treatment is available to manage symptoms and slow the progression of the disease. Treatment options may include medications, such as ursodeoxycholic acid (UDCA), to improve bile flow and protect the liver, and other immunosuppressive drugs to control the autoimmune response.
It is important to catch and treat PBC early to prevent liver damage and avoid complications such as cirrhosis, liver failure, and liver cancer. If you are experiencing symptoms or have a family history of liver disease, it is important to speak with your doctor for an accurate diagnosis and appropriate treatment plan.
WHAT CAN I DO AT HOME TO PREVENT MORE LIVER INJURY?
WHAT TREATMENTS SHOULD I DISCUSS WITH MY DOCTOR?
At each clinic visit you should consider discussing the following items with your healthcare provider:
If your liver tests are normal. Decades of evidence has shown that people with PBC who have abnormal liver tests are more likely to develop cirrhosis and need liver transplant. If your tests are abnormal, you should discuss medications including ursodiol (ursodeoxycholic acid), obeticholic acid, or a 'fibrate' medication.
What symptoms you are feeling. Your doctor can prescribe medications that could help with itching as well as discuss strategies to manage dry eyes and dry mouth, two symptoms commonly seen with PBC.
Your alcohol intake and strategies to quit or minimize alcohol use to prevent additional liver damage.
Your weight and strategies to achieve and maintain a healthy weight to prevent additional liver damage.
Vaccinations. In general, individuals with liver disease should receive vaccinations for hepatitis A and B, TDAP, yearly influenza vaccination, and be up to date with COVID vaccinations. Individuals with cirrhosis should also receive pneumonia vaccinations. A complete list of recommended vaccinations can be obtained from the CDC website on Vaccinations for those with liver disease. Any of these infections can cause your liver to become much sicker if you are not appropriately protected from vaccination.
WHAT RESOURCES ARE AVAILABLE TO HELP ME?
This link provides a "patient friendly" document that summarizes the diagnosis and management of alcohol-associated liver disease to help you better understand your condition and the care you need. It is provided by the AASLD (American Association for the Study of Liver Diseases), a well known organization that provides guidelines that doctors frequently reference.
