From a clinical point of view: Questions and answers about primary galcholangitis (PBC)

What is PBC?

Primary Galcholangitis (PBC) occurs when your own immune system starts to respond to you and focuses on the small bile ducts in the liver. These attacks lead to an accumulation of bile and a blockade of bile stream (cholestasis), causing the bile ducts to be inflamed and the liver cells were damaged. This damage results in scars, 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 do not understand all contributing factors, but a family history of PBC or immune disease is probably the most important. Traditionally it was thought that PBC was a disease that only hit white middle -aged women, but we find PBC in men, people from different breeds and younger and older people too. However, PBC is still more common in women than for men.

What are the symptoms of PBC?

Many people with PBC have no symptoms that are specific to the disease, and when they do that, the symptoms vary from person to person. The most common initial symptoms are itching (called itching) and fatigue. People with PBC can also report abdominal pain; darkening 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 can be SICCA syndrome that is chronic dryness of the eyes and mouth, and increased cholesterol levels.

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 healthcare provider (HCP).

Read: Living with primary Gal Cholangitis >>

How is PBC diagnosed?

HCPs can initially have difficulty set a PBC diagnosis, because the disease cannot show with non-specific symptoms such as itching and fatigue. But there are different ways to diagnose PBC.

Check blood tests for increased levels of an enzyme called alkalic phosphatase and bilirubin, as well as an immune indicator called an antimitochondrial antibody.

PBC is diagnosed when a person has blood tests that increased alkaline phosphatase levels shows as a result of changes in bile current (called cholestase), 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 performed to diagnose the condition if there is care for liver damage (based on high bilirubin levels).

What are primary bile ducts cholangitis treatments?

Various medicines have been approved to help control PBC. Most treatment plans start with first -line therapy, which means it is the first medication to try your HCP. Your doctor usually expects an improvement in blood test to confirm that the medicine works. If there is insufficient biochemical response to first -line therapy after 12 months, your doctor may consider additional treatment options. In some cases, evaluation for other therapies on the basis of clinical judgment and individual blood tests can take place between 6 and 12 months. Second line treatments, which can be used in combination with primaryine options or alone, have demonstrated improvements in liver enzyme levels and can help relieve the symptoms such as itching. Treatment decisions must always be made by your HCP based on your specific clinical profile.

Are there changes in lifestyle that can be useful to keep your health during life with PBC?

Every patient must discuss treatment and care approaches with his HCP, but in general we encourage people with PBC to concentrate on a healthy lifestyle, including regular exercise and a diet rich in entire foods, fiber and lean proteins. People who live with PBC must also concentrate on their bone health because osteopenia And osteoporosis are more common in people with PBC. Stopping smoking and limiting alcohol consumption are also recommended.

Read: Living with primary Gal Cholangitis >>

What are some tips to stay on top of your health if you have PBC?

First, you must be your own health care lawyer. Know which medicine you use and whether it works. This is really difficult for PBC because there have been significant changes in what we define as a “good treatment reaction”. In other words, make sure you and your doctor stay up -to -date. Make sure you find a provider who listens to what you say and is on a common basis for treatment goals. Your liver tests will help determine whether the treatment works, so make sure you understand where you and your HCP want these figures to be.

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

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

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