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Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a progressive liver disease. If left untreated, it may cause liver failure. After receiving a PBC diagnosis, you’ll want to know how this condition may affect your life expectancy (how long you’re expected to live).
Your doctor may be able to predict your prognosis (outcome) by looking at several factors, including:
Continue reading to learn more about how each of these factors can help predict your life expectancy and prognosis with PBC.
PBC is an autoimmune condition that can gradually get worse. It can progress through four stages. Stages 1 and 2 are early stages of PBC, when you have inflammation and fibrosis (thickened tissue caused by scarring) in the area around the portal vein (a vein that carries blood to your liver). Stage 3 is defined by changes in the structure of the liver from scarring. Stage 4 is characterized by cirrhosis — severe liver scarring that can lead to liver damage and liver failure.
Doctors may use your PBC stage, in addition to other factors related to your condition and overall health, when predicting your outcome and deciding your treatment plan.
Liver function tests are blood tests that can check the health of your liver. They measure the levels of substances that are made by the liver and found in your blood. Your doctor may use the results of these tests to predict the course of your PBC and how you might respond to treatment. However, it’s important to remember that no single test tells the whole story. Your doctor will look at your results over time, since trends in your lab values usually give a clearer picture than one reading alone.
Bilirubin levels are among the best ways to tell how long someone with PBC might live and how they will do over time. The liver makes bilirubin, which is a waste product that’s left over when red blood cells break down. It’s measured in milligrams per deciliter, and the normal range for adults is between 0.2 and 1.3 milligrams per deciliter.
Older medical models used bilirubin levels to estimate the average survival time (the time a person lives after a PBC diagnosis) as:
Some researchers believe this model is too basic and shouldn’t be the only factor used to predict the course of PBC. It’s also worth noting that bilirubin levels might stay normal early on in PBC, which makes them a less helpful metric at that stage.
Alkaline phosphatase (ALP) is an enzyme (a type of protein) that’s found throughout the body, but mainly in your liver. High levels of ALP can be a sign of liver problems. Lower ALP levels are associated with better outcomes in PBC, a lower risk of death, and increased length of time living with PBC without needing a liver transplant.
Your ALP level can also help predict how treatment might work for you. People whose ALP is less than twice the normal limit are more likely to do well with a medicine called ursodiol (also known as ursodeoxycholic acid or UDCA), which is the first treatment for PBC.
While individual lab values, like bilirubin and ALP, can give healthcare providers important clues about your prognosis, they don’t tell the whole story. To get a more accurate picture of your life expectancy and prognosis with PBC, doctors may use a model that considers several different lab values together.
A gastroenterologist (a specialist in conditions of the digestive system) may use a model to predict how well you’ll respond to treatment, your risk of needing a liver transplant, or your risk of liver-related death. Currently, there are two predictive models that healthcare providers use — the GLOBE score and the UK-PBC score. These models are tools to help predict your prognosis using specific information, such as:
These models can give your gastroenterologist a clearer picture of your outlook to help you make personalized decisions about your care with PBC.
PBC symptoms (or lack of symptoms) can help predict your life expectancy and prognosis. In general, more severe symptoms in the early stages of PBC may predict a faster disease progression.
People who are asymptomatic (don’t have symptoms) are often in an early stage of the disease. Between 50 percent and 70 percent of people with asymptomatic PBC are expected to live at least 10 years without treatment. About 1 in 4 people with asymptomatic PBC will progress to liver failure within 10 years.
People who are symptomatic (have PBC symptoms) are usually in a later stage of PBC. Without treatment, half of the people with symptomatic PBC are expected to live between five and eight years.
Researchers aren’t sure which factors increase your likelihood of developing PBC symptoms. People with PBC who never develop symptoms will likely have the same life expectancy as the general population.
The most common early symptoms of PBC are fatigue and pruritus (itching). Fatigue is often considered the most debilitating symptom of PBC and can significantly decrease your quality of life. However, more severe fatigue and itching aren’t always associated with more severe PBC.
With appropriate treatment, the life expectancy for people with PBC can be the same as that of the general population or people without PBC. It’s important to work with your healthcare provider to get treatment for PBC to prevent it from worsening and to extend your life expectancy.
Ursodiol is the first treatment used for people with PBC. It can help slow the progression of PBC and decrease the need for a liver transplant. Ursodiol is more effective at preventing the progression of PBC in people with stage 1 or stage 2 PBC.
People with a good response to ursodiol in the first six months to two years usually have a better prognosis. Between 25 percent and 30 percent of people with PBC taking ursodiol have a complete response to this treatment, according to an article in the journal Hepatic Medicine. A complete response means that PBC doesn’t progress to the next stage, and all liver function test results are normal.
People with a good response to ursodiol can live as long as healthy people without PBC. A 2005 study examined the survival rate of more than 250 people with PBC treated with ursodiol. Researchers found that those treated with ursodiol in the early stages of PBC had a survival rate similar to the general population’s.
Elafibranor (Iqirvo) and seladelpar (Livdelzi) are newer PBC treatments. They may be taken alone or in combination with ursodiol.
In clinical trials involving people with PBC, elafibranor and seladelpar were shown to reduce ALP levels, a marker of liver function. Researchers don’t yet know if the drugs improve survival in people with PBC, but they are doing more tests in clinical trials.
If other treatment options don’t stop the progression of PBC, you may need a liver transplant. Liver transplantation is the only effective treatment for people with liver failure due to PBC. A liver transplant involves surgery to replace your diseased liver with a healthy liver from a living or deceased donor.
The article from Hepatic Medicine states that about 85 percent of people who receive a liver transplant live at least five more years. PBC can come back in about 20 percent of people three to seven years after they receive a new liver. However, it can still be managed with treatment and monitoring.
It’s important to talk with your healthcare provider about tools like the GLOBE score and the UK-PBC score. These tools help determine your risks and make medical choices easier. Don’t be afraid to ask questions or speak up if something is unclear. Your doctor is there to help you understand, not just to give treatments. Working together helps you stay healthier while living with PBC.
On myPBCteam, people share their experiences with primary biliary cholangitis, get advice, and find support from others who understand.
How did your healthcare team discuss your PBC prognosis with you? Let others know in the comments below.
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Recently Diagnosed With PBC. Takjng UDCA Enzymes Coming Down. Fibroscan PDa Is 23. Asymptomatic Bilibrum,Albumin Normal. Life Expect
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