Facts About PBC

Facts About PBC

A diagnosis of Primary Biliary Cholangitis (formerly known as primary biliary cirrhosis) (PBC) can often be stressful, due to misunderstandings about what the disease is and the ways in which the liver can become damaged. By understanding more about PBC, you will be able to manage your health proactively and better communicate with your doctor.

What causes PBC?

PBC is a rare liver disease most likely caused by an autoimmune reaction that damages bile ducts – tube-like structures that carry bile from the liver to the intestine to help with the digestion of food. When the bile ducts are damaged bile is not able to flow through them normally and the bile collects in the liver and causes damage.

The causes of PBC remain unknown, but evidence suggests it could be genetic or caused by undetermined environmental factors.

While PBC is a disease of the liver, it is important to note that it is not caused by alcohol consumption.

Who is at risk for PBC?

It is estimated that 90 percent of people who are diagnosed with PBC are women, and many people who are diagnosed are between 40 and 60 years old. It is important to note, however, that men and younger women can also develop PBC.

How is PBC diagnosed?

Doctors can diagnose PBC by taking blood samples and measuring certain biochemical markers within that sample that are related to liver function. Often times, certain elevations can indicate areas of concern.

These tests can show if there are elevated levels of alkaline phosphatase (ALP) and if anti-mitochondrial antibodies (AMAs) are present – these markers can be indicators of PBC. Some patients will also have a liver biopsy performed to help establish the diagnosis or determine the state of disease.

PBC is most often diagnosed in people who are not showing any symptoms.

Is PBC an aggressive, fast-moving disease?

While not aggressive, PBC is a progressive disease, meaning the damage can get worse over a long period of time. To better explain the progression, clinicians have established the “Four Stages of PBC”:

Stage 1 – Portal Stage: Identified by florid duct lesion, which means medium-sized bile ducts show intense chronic inflammation and damage to their walls.

Stage 2 – Periportal Stage: Disease progression continues when proliferation of the small bile ducts begin. This indicates obstruction of the small, tube-like structures that carry bile from the liver to the small intestines.

Stage 3 – Septal Stage: Appearance of fibrous septa – excess tissue – and scarring indicates the disease is moving toward cirrhosis.

Stage 4 – Cirrhosis (scarring) of the liver occurs, causing permanent damage. With early diagnosis of cirrhosis, the damage can be limited and managed.

What is my prognosis?

PBC is classified as a chronic liver disease, which means that it is a long-lasting condition that can be controlled but not cured.

The prognosis of people with PBC has improved over the last few decades due to earlier diagnosis and improved treatment. Different people have different experiences with the disease – the rate of damage and speed of progression vary from person to person.

Evidence suggests that early access to treatment delays progression in PBC, and the outcomes of patients taking treatment is better than those who are untreated.


Sources:

Lee, Y.M., Kaplan M.M. The natural history of PBC: has it changed? Semin. Liver Dis. 2005 Aug;25(3):321-6.

Primary Biliary Cirrhosis. U.S. National Institutes of Health, National Institute of Diabetes and Digestive and Kidney Diseases.

Kumagi T, Healthcote E.J. Primary biliary cirrhosis. Orphanet Journal of Rare Diseases. 2008; 3:1. doi:10.1186/1750-1172-3-1.

Pathologic Features and Evolution of Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis.” Scheuer, Peter J. Mayo Clinic Proceedings, Volume 73, Issue 2, 179-183.

 

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