How Is Primary Biliary Cholangitis Detected and Managed?

Primary biliary cholangitis (PBC) is an ongoing autoimmune disorder that causes damage and swelling in the small bile ducts within the liver. In the event that primary biliary cholangitis goes untreated or undetected, it may cause significant scar tissue buildup in the liver, liver failure, and the need for a liver transplant, making it vital to recognize the warning signs and risk factors associated with the condition. PBC was previously known as "primary biliary cirrhosis." However, in 2015, PBC patient advocacy groups and medical experts decided to change the name. A number of people diagnosed with primary biliary cholangitis thought that utilizing the word cirrhosis conveyed an erroneous association with alcohol use and was deceptive given that many individuals living with primary biliary cholangitis are not affected by extreme liver damage (liver cirrhosis).

Am I at risk for developing primary biliary cholangitis?

Women who are middle-aged are most likely to develop primary biliary cholangitis. As a matter of fact, it is estimated that the condition impacts one out of every 1,000 women over the age of 40. While men can also develop the disease, approximately 90% of individuals having PBC are women. PBC can run in families, and an individual might be at an increased risk if they have a sibling, parent, or child who is also affected by the liver condition. Additionally, a minimum of half of those with primary biliary cholangitis have also been diagnosed with another autoimmune condition. Patients affected by health issues, such as Sjogren's syndrome, Raynaud's phenomenon, and autoimmune thyroid disease, should be even more mindful of their health risks. The root cause of primary biliary cholangitis remains unknown, but the liver condition is not caused by consuming alcohol and is not transmissible to others.

Are there any warning signs of primary biliary cholangitis?

Primary biliary cholangitis can take many years to present symptoms. Furthermore, many symptoms of PBC can mimic those of other medical conditions, causing it to be more difficult for physicians to diagnose. Commonly, individuals who have PBC are diagnosed when evidence of liver damage is detected during standard lab work. Routine laboratory analysis is a standard way to assess the function of the liver and may include evaluating factors, such as:

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

Among those who have signs or indications of primary biliary cholangitis, the most frequently reported symptoms are itching (pruritus) and exhaustion.

How is PBC detected?

One of the vital indicators doctors first review to diagnose PBC is the body's ALP (alkaline phosphatase) level.

• During the early stages of PBC, abnormally high alkaline phosphatase could be an early indication that primary biliary cholangitis might have developed.
• Alkaline phosphatase, which is at times called "alk phos,” is an enzyme present in the body.
• An unusually increased ALP level could point to damage in the liver.

It is also necessary to test for AMAs (antimitochondrial antibodies) in instances where primary biliary cholangitis is likely.

• The appearance of antimitochondrial antibodies can signal primary biliary cholangitis.
• AMAs are substances produced by the body's immune system.

Some doctors conduct a liver biopsy to verify a diagnosis of PBC and to evaluate how far the disease has advanced. But liver biopsies usually are not necessary to render a PBC diagnosis.

How is PBC treated?

To date, there is no way to reverse PBC; however, two medications have been approved in the United States to assist in managing the condition and minimizing the further advancement of liver damage. Healthcare guidelines advocate that physicians evaluate ALP amounts in patients with PBC on a 3 to 6–month basis to examine disease progression and determine to what extent medication therapy is helping. Since primary biliary cholangitis is a rare medical disease, a large number of patients get treatment from gastroenterologists and liver specialists who have extensive training in managing conditions involving the liver.

Find help for primary biliary cholangitis in DFW

September is PBC Awareness Month, and Sunday, September 12th is International PBC Day. If you believe you or a family member may have primary biliary cholangitis, now is a great time to learn more and explore your options. PBC Awareness Month also provides an opportunity for people who have the liver issue to celebrate the triumphs of the PBC community and further their understanding of the disease. Reach out to Texas Digestive Disease Consultants today to schedule a visit with one of our GI doctors and determine if alkaline phosphatase testing is right for you. We treat patients throughout the DFW area who have PBC and can help you manage this health condition.