What to Know About Primary Biliary Cholangitis (PBC)

Referred to as a progressive autoimmune disease of the liver, primary biliary cholangitis (PBC) leads to damage and enlargement of the small bile ducts in the liver. In the event that the disease is not detected or treated, it could cause serious liver scarring, hepatic failure, and liver transplant needs, making it crucial to be aware of the indications and risks factors surrounding the condition. The condition was formerly known as "primary biliary cirrhosis" but PBC medical experts and patient advocacy groups decided to change the name in 2015. A number of patients impacted by the disease felt that using the word cirrhosis caused an erroneous association with alcohol consumption and was commonly misleading since most individuals diagnosed with PBC do not have liver cirrhosis (advanced liver damage).

Who is at risk for PBC?

Women over the age of 40 are most likely to develop primary biliary cholangitis. It is estimated that the condition impacts one in 1,000 women over the age of 40. While men can develop the disease as well, nearly 90% of people diagnosed with PBC are women. Primary biliary cholangitis can run in families, and a person might be at an increased risk if they have a sibling, parent, or child who has been diagnosed with the liver disease. Likewise, at least 50% of individuals with PBC also have another autoimmune condition. People who are living with health problems, like Raynaud's syndrome, autoimmune thyroid disease, and Sjogren's syndrome, need to be especially mindful of their condition and general health. The specific source of primary biliary cholangitis is unknown, but it is not caused by drinking alcohol and is not communicable.

The signs of primary biliary cholangitis

Among some patients, primary biliary cholangitis can take several years to present symptoms. Several symptoms of the disease are a lot like those of other health concerns, making it more difficult for physicians to identify. Oftentimes, patients who have primary biliary cholangitis get diagnosed due to evidence of liver damage detected during routine liver labs. Regular lab tests are a standard way to assess liver function and commonly include the evaluation of:

• albumin
• gamma-glutamyl transpeptidase (GGT)
• prothrombin time (PT)
• aspartate aminotransferase (AST)
• alkaline phosphatase (ALP)
• bilirubin
• alanine aminotransferase (ALT)

Within patient populations who experience symptoms of PBC, those most frequently reported include diminished energy and pruritus (itching).

How is PBC diagnosed?

The critical markers doctors first assess to diagnose PBC is a patient's ALP (alkaline phosphatase) readings.

• In the early stages of primary biliary cholangitis, unusually elevated alkaline phosphatase could serve as an early indication to physicians that primary biliary cholangitis may be present.
• ALP, sometimes referred to as "alk phos,” is an enzyme found in the body.
• An unusually high ALP level can indicate damage to the liver.

Furthermore, it is necessary to test for antimitochondrial antibodies (AMAs) if primary biliary cholangitis is likely.

• The presence of antimitochondrial antibodies is an indicator of primary biliary cholangitis.
• Antimitochondrial antibodies are immunoglobulins produced by the immune system.

In some instances, physicians conduct a biopsy of the liver to establish a PBC diagnosis and to assess disease advancement. But liver biopsies are generally not necessary to diagnose the liver condition.

How is primary biliary cholangitis treated?

There is currently no way to reverse primary biliary cholangitis, but two medications have been approved in the United States to aid in managing the condition and slow the further progression of damage in the liver. Healthcare recommendations advocate that medical professionals examine alkaline phosphatase levels in patients with primary biliary cholangitis every 3 to 6 months to assess the progression of the disease and ascertain to what extent medication therapy is working. Because primary biliary cholangitis is a rare health condition, a large number of people turn to gastrointestinal doctors and liver specialists who have advanced training and expertise in treating liver diseases.

Find hope and support in your Indianapolis, IN community

September is Primary Biliary Cholangitis Awareness Month, and Sunday, September 12th is International PBC Day. This provides a chance for people who have been diagnosed with the liver problem to pay homage to the achievements of the primary biliary cholangitis patient community and spread further awareness about this liver condition. In the event you think you or a family member could be living with primary biliary cholangitis, we urge you to contact Indianapolis Gastroenterology and Hepatology today. We can help you arrange an appointment with one of our Indianapolis, IN gastroenterologists to learn about your primary biliary cholangitis risk factors and details about ALP testing.