Facts About Primary Biliary Cholangitis (PBC)

What is PBC?

Primary biliary cholangitis (PBC) occurs when your own immune system starts to react against you and targets the small bile ducts in the liver. These attacks lead to a buildup of bile and a blockage of bile flow (cholestasis), leading to the bile ducts becoming inflamed and the liver cells damaged. This damage results in scarring, called fibrosis. Over time, scar tissue replaces healthy tissue (called cirrhosis) and the bile ducts are destroyed, which causes damage to the liver that gets worse over time.

What are the risk factors for PBC?

We don’t understand all of the contributing factors, but a family history of PBC or immune disease is probably the most important. Traditionally, PBC was thought to be a disease that only affected middle-aged white women, but we are finding PBC in men, people of various races, and younger and older people as well. However, PBC is still more prevalent in women than men.

What are the symptoms of PBC?

Many people with PBC have no symptoms that are specific to the disease, and when they do, the symptoms vary from person to person.The most common initial symptoms are itching (called pruritus) and fatigue. People with PBC may also report abdominal pain; darkening of the skin; small yellow or white bumps under the skin (xanthomas) or around the eyes (xanthelasmas); dry mouth and eyes; and bone, muscle and joint pain.

Other signs and symptoms may include sicca syndrome, which is chronic dryness of the eyes and mouth, and elevated cholesterol levels.

Many people with PBC do not have symptoms other than itching and fatigue in the early stages of the disease. If you’re having any symptoms of PBC, discuss them with your healthcare provider (HCP).

Read: Living with Primary Biliary Cholangitis >>

How is PBC diagnosed?

HCPs may have difficulty making a PBC diagnosis initially since the disease can present with non-specific symptoms like itching and fatigue. But there are several ways to diagnose PBC.

Blood tests check for increased levels of an enzyme called alkaline phosphatase and bilirubin, as well as an immune indicator called an antimitochondrial antibody.

PBC is diagnosed when a person has blood work that shows elevated alkaline phosphatase levels due to changes in bile flow (called cholestasis) as well as the presence of the antimitochondrial antibody.

If the results of the blood tests are unclear, a liver biopsy or other immune tests can also be done to diagnose the condition if there is concern for liver damage (based on high bilirubin levels).

What are primary biliary cholangitis treatments?

There are several medications approved to help control PBC. Most treatment plans start with the first-line therapy, which means it’s the first medication your HCP will try. Your doctor will typically expect to see an improvement in blood work to confirm the medication is working. If there is an inadequate biochemical response to first-line therapy after 12 months, your doctor may consider additional treatment options. In some cases, based on clinical judgment and individual blood work trends, evaluation for other therapies may occur between 6 and 12 months. Second-line treatments, which can be used in combination with first-line options or alone, have shown improvements in liver enzyme levels and may help ease symptoms, such as itching. Treatment decisions should always be made by your HCP based on your specific clinical profile.

Are there lifestyle changes that may be helpful for maintaining your health while living with PBC?

Each patient should discuss treatment and care approaches with their HCP, but in general, we encourage people with PBC to focus on a healthy lifestyle, including regular exercise and a diet rich in whole foods, fiber and lean proteins. People living with PBC should also concentrate on their bone health because osteopenia and osteoporosis are more common in people with PBC. Quitting smoking and limiting alcohol consumption are also recommended.

Read: Living with Primary Biliary Cholangitis >>

What are some tips for staying on top of your health if you have PBC?

First, you need to be your own health advocate. Know what medication you’re taking and if it’s working. This is really hard for PBC because there have been significant changes in what we define as a “good treatment response.” In other words, make sure you and your doctor are staying up to date. Be sure you find a provider that is listening to what you say and is on common ground for treatment goals. Your liver tests are going to help determine whether treatment is working, so be sure to understand where you and your HCP want these numbers to be.

You should also have your thyroid function checked once a year and keep track of your bone health because of the increased risk of osteopenia and osteoporosis.

Living with PBC can also be emotionally challenging. So, a strong support system is important to help maintain mental health. In addition to family and friends, patient advocacy groups and online communities can offer comfort and advice and emotional support.

Last, talk to your family. Although not firmly established in current guidelines, we encourage discussing screening with your HCP, especially for first degree family members (sisters, daughters, mothers) because we know they are at increased risk. Although PBC is less common in men, they should still talk to their HCP if they have symptoms.