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Primary biliary cholangitis (PBC), formerly called primary biliary cirrhosis, is a progressive liver disease that affects the liver’s bile ducts. If left untreated, it may cause liver failure. After receiving a diagnosis of PBC, you’ll want to understand how this condition may progress and, ultimately, how it might 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 living with this chronic condition.
Primary biliary cholangitis is an autoimmune disease that can gradually get worse. Progression rates vary significantly among individuals with the condition. It can progress through four stages:
Doctors may use your PBC stage, in addition to other factors related to your condition and overall health, when predicting your outcome and recommending a 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 gastroenterology specialist (digestive system doctor) may use the results of these tests to help 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.
The liver makes bilirubin, which is a waste product that’s left over when red blood cells break down. Bilirubin levels have historically been an important predictor of how long someone with PBC might live and how they will do over time. Older medical models used bilirubin levels alone to estimate average survival time (how long a person will live with a health condition).
Bilirubin levels are measured in milligrams per deciliter, and the normal range for adults is 0.2 to 1.3 milligrams per deciliter. Bilirubin levels might remain within the normal range early on in PBC, which makes them a less helpful metric at that stage.
While bilirubin alone remains an important predictor, predictive tools have improved significantly in recent years, and specialists now use complex models that incorporate bilirubin levels and have been developed to work closely with current treatment protocols.
Alkaline phosphatase (ALP) is an enzyme (a type of protein) that’s found throughout the body, but mainly in the 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. One study showed more likely treatment success among people whose ALP was less than 1.67 times the normal limit six months after taking ursodeoxycholic acid (UDCA, also known as ursodiol), which is the first treatment for PBC.
While individual lab values, like bilirubin and ALP, can give healthcare providers important clues about prognosis, they don’t tell the whole story. To get a more accurate picture of a person’s life expectancy and prognosis with PBC, doctors today more commonly use a model that considers several different lab values together.
A gastroenterologist 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, healthcare providers use two predictive models, 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 treatment decisions about your care with PBC.
PBC symptoms (or lack of symptoms) can help predict your prognosis and life expectancy. In general, more severe symptoms in the early stages of PBC may predict a faster disease progression. These symptoms can include:
However, PBC is increasingly diagnosed before it becomes symptomatic (causes PBC symptoms). More than half of people diagnosed are asymptomatic (don’t have symptoms). This has allowed for earlier treatment and life expectancies approaching the normal range.
People who are asymptomatic are often in an early stage of the disease. About half of asymptomatic people will develop symptoms within five to 10 years. People who are symptomatic are usually in a later stage of PBC.
Today, with standard therapy (UDCA), liver-transplant-free survival rates have increased significantly. One study found 10-year liver-transplant-free survival rates approaching 80 percent among people treated with UDCA. This means that after 10 years, 80 percent of the people treated with UDCA who didn’t get a liver transplant were still alive.
Researchers aren’t sure which factors increase a person’s 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 primary biliary cholangitis are fatigue and pruritus. Fatigue is often considered the most debilitating symptom of PBC and can significantly reduce 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 early and effective treatment for PBC to prevent it from worsening and to extend your life expectancy.
UDCA 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. UDCA is more effective at preventing the progression of PBC in people with stage 1 or stage 2 PBC.
People with a good response to UDCA in the first six months to two years usually have a better prognosis. At least 25 percent to 30 percent of people with PBC taking UDCA have a complete response to this treatment, according to research published 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 PBC who respond well to UDCA have the same life expectancy as the general population.
Elafibranor (Iqirvo) and seladelpar (Livdelzi) are newer PBC treatments. They’re both peroxisome proliferator-activated receptor (PPAR) agonists, which are thought to work by activating proteins in liver cells that help regulate inflammation, bile acid production, and fat metabolism. By targeting these pathways, PPAR agonists can reduce bile acid buildup and liver inflammation, which may help slow liver damage in people with PBC. They may be taken alone or in combination with UDCA.
In clinical trials, elafibranor and seladelpar were shown to reduce ALP levels, a key marker of liver function in people with PBC. 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 a person’s diseased liver with a healthy liver from a living or deceased donor.
Due to the effectiveness of first-line treatment with UDCA and other therapies, the frequency of liver transplants for people with PBC has been decreasing. About 80 percent of people who receive a liver transplant live at least 10 more years. PBC comes back in about 20 percent to 30 percent of people within 10 years after they receive a new liver, with most recurrences between three and six years after transplantation. However, the liver disease usually progresses more slowly the second time and 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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