The primary gallcholangitis (PBC) is an autoimmune disease that mainly affects women and people who have been assigned to women at birth (Afab). PBC damages the liver and, if you remain untreated, can lead to serious problems such as cirrhosis and liver failure.
The good news is that treatments are available that can help reduce the symptoms and slow down the progression of the disease.
We turned to Nancy Reau, MD, Associate Director of Solid Organ Transplantation and Section of Hepatology at Rush University Medical Center to get the facts and tips for managing PBC.
What is PBC?
Primary gallcholangitis (PBC) occurs when your own immune system reacts against you and aims the small bile ducts in the liver. These attacks lead to a bile building and a blocking of the bile flow (cholestasis), which means that the bile ducts ignite and the liver cells are damaged. This damage leads to scars called fibrosis. Over time, scar tissue replaces a healthy tissue (referred to as cirrhosis) and the bile ducts are destroyed, causing the liver that worsen over time.
What are the risk factors for PBC?
We don’t understand all factors, but a family history of PBC or immune diseases is probably the most important. Traditionally it was assumed that PBC is a disease in which only white women were affected by the middle of the age, but we find PBC in men, people of different breeds as well as younger and older people. However, PBC is still more common in women than men.
What are the symptoms of PBC?
Many people with PBC have no symptoms that are specific to the disease, and if this is the case, the symptoms vary from person to person. The most common initial symptoms are itching (called pruritus) and fatigue. People with PBC can also report abdominal pain. Darkening of the skin; Small yellow or white bumps under the skin (Xanthomas) or around the eyes (Xanthelasms); Dry mouth and eyes; And bones, muscles and joint pain.
Other signs and symptoms can include the Sicca syndrome, in which the eyes and mouth are chronic drought, and the increased cholesterol level.
Many people with PBC have no other symptoms than itching and fatigue in the early stages of the disease. If you have symptoms of PBC, discuss them with your health service provider (HCP).
Reading: Living with primary gallcholangitis >>
How is PBC diagnosed?
HCPS may initially have difficulty making a PBC diagnosis, since the disease cannot have specific symptoms such as itching and tiredness. However, there are various ways to diagnose PBC.
Blood tests check for an increased mirror of an enzyme, which are called alkaline phosphatase and bilirubin, as well as an immunindicist that is referred to as antimitochondria.
PBC is diagnosed when a person has an ancestral work, which has increased alkaline phosphateSESEPIEGE due to changes in the bile flow (as cholestasis) and the presence of the antimitochondria antibody.
If the results of the blood tests are unclear, a liver biopsy or other immune tests can also be carried out in order to diagnose the condition if liver damage concerns (based on high bilirubin mirrors).
What are primary gall -cholangitis treatments?
Several medication are approved to control the PBC. Most treatment plans begin with first line therapy, which means that it is the first medication to try out your HCP. Your doctor usually expects an improvement in the blood test to confirm that the medication works. If there is an inadequate biochemical reaction to first-line therapy after 12 months, your doctor can consider additional treatment options. In some cases, the evaluation for other therapies between 6 and 12 months can occur on the basis of the clinical judgment and the individual blood test trends. The second line treatments that can be used in combination with first line options or alone have shown improvements in the liver enzyme level and can help relieve symptoms such as itching. Treatment decisions should always be made by your HCP based on your specific clinical profile.
Are there any changes to the lifestyle that may be helpful to maintain your health when living at PBC?
Every patient should discuss treatment and care approaches with their HCP, but in general we humans with PBC encourage themselves to concentrate on a healthy lifestyle, including regular exercise and a diet that is rich in full-scale, fiber and lean proteins. People who live with PBC should also focus on their bone health because osteopenia and osteoporosis are more common in people with PBC. It is also recommended to stop smoking and to limit alcohol consumption.
Reading: Living with primary gallcholangitis >>
What are some tips to stop your health if you have PBC?
First you have to be your own health lawyer. You know which medication you take and whether it works. This is really difficult for PBC because there have been significant changes in relation to what we define as a “good treatment reaction”. In other words, make sure that you and your doctor will stay up to date. Make sure you find a provider who listens to the treatment goals, what you say and stand on the common reason. Your liver tests will help to determine whether the treatment works. So understand where you and your HCP want to have these numbers.
You should also have your thyroid function checked once a year and keep an eye on your bone health because the risk of osteopenia and osteoporosis is increased.
Life with PBC can also be emotionally challenging. A strong support system is therefore important to maintain mental health. In addition to family members and friends, patient representative groups and online communities can offer comfort and advice and emotional support.
Talk to your family last. Although we are not firmly established in the current guidelines, we recommend discussing screening with your HCP, especially for family members (sisters, daughters, mothers) first degree because we know that you have an increased risk. Although PBC is less common in men, you should still speak to your HCP if you have symptoms.
This educational resource was created with the support of Gilead.
US-Onbc-2765 06/25
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