4 things to know about primary biliary cholangitis (PBC)
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic autoimmune disease. Researchers estimate that about 65 out of every 100,000 women in the United States have PBC; this number is much lower in men. When a person has PBC, the immune system attacks the small bile ducts, which can cause damage to the liver.
It can be scary to receive a PBC diagnosis, but you’re not alone. We share four things to know about the disease, including key symptoms and a few valuable resources.
PBC is considered an autoimmune disease
PBC is considered an incurable, long-term autoimmune disease. This means the body's immune system is mistakenly attacking healthy cells and tissue. Researchers believe that PBC may be caused by some combination of genetic and environmental risk factors, but are not sure of the exact root of the disease.
Genetics do play a major role in autoimmune disorders, and many people diagnosed with one autoimmune condition may eventually be diagnosed with others. In an interview with the American Liver Foundation, Dr. Craig Lammert, an expert on autoimmune liver diseases at Indiana University School of Medicine, said that “... good rule of thumb is that at least half of all patients with PBC will have at least one other autoimmune condition. Of this group, about 10% to 20% will have two or more additional autoimmune conditions.”
Many autoimmune conditions can have overlapping symptoms, so it’s best to work with your healthcare team to get a clearer picture of your diagnoses and treatment regimens.
People with PBC may not have any noticeable symptoms at diagnosis
Many people with PBC do not have any noticeable symptoms when diagnosed. The disease may be diagnosed when blood tests are done for other reasons, such as routine testing, or with a liver biopsy.
When symptoms do appear, the most common ones amongst PBC patients include fatigue and itchy skin:
- According to the American Association for the Study of Liver Diseases (AASLD), fatigue has been found in 50-78% of PBC patients. Fatigue from PBC can be constant or slowly progressive.
- Early studies showed that itching occurs for 20-70% of PBC patients. Since a growing number of patients with PBC are diagnosed early, before symptoms appear, the percentage of patients reporting itching is lower. The severity of itching in PBC can fluctuate over time.
There is no cure for PBC, but there are treatment options
Unfortunately, there is no cure for PBC, but there is medication available to treat the disease itself and key symptoms. If none of these work for you, speak to your doctor about off-label options, too.
- Ursodeoxycholic acid (UDCA) is a medication that helps move bile through your liver. It can improve liver function and reduce liver scarring. It's less likely to help with symptoms like itching and fatigue.
- Obeticholic acid (Ocaliva) is a medication that works by improving bile flow and reducing inflammation. It may be offered as an option for treating PBC, either in combination with UDCA (if UDCA is not working well enough) or on its own (for people who cannot take UDCA).
- Liver transplant is a procedure that may help prolong your life when medications no longer control PBC and the liver begins to fail. Liver transplants have good long-term outcomes for people with PBC, but sometimes the disease comes back several years later in the transplanted liver.
- Treatment for fatigue: Daily habits, diet and exercise, and other health conditions can affect how tired you feel, so working on a plan to ensure you are taking care of yourself in these categories is critical.
- Treatment for itching: Cholestyramine, colestipol, and colesevelam (Anion-Exchange Resins) are resins that bind to negatively charged anions, such as bile acids. They have a long history of clinical use when it comes to itching. Some small clinical trials have shown that Rifampicin, a pregnane X receptor agonist, has been effective at treating itching in patients with PBC. Opioid antagonists may also reduce an itching sensation.
There are resources for people living with PBC and their families
People living with PBC, their loved ones, and their care partners are not alone. There are a variety of PBC resources and support networks out there:
- PBCers: The PBCers Organizations’ mission is to offer education and support to PBC patients, family members and friends, and to raise funds to help research the causes and cure for PBC. They have local contacts and group meetings, with the aim of helping one another on their respective PBC journeys.
- Living with PBC highlights individual PBC stories, shares information about events, and has a video hub.
- Primary Biliary Cholangitis Facebook group: This is a private group with over 4,000 members discussing their experiences with PBC.
- American Liver Foundation: The American Liver Foundation is a national organization for patients, families, and professionals that is dedicated to understanding liver diseases. They have an array of resources, including events, clinical trial opportunities, a video library, and blogs.
- PBC Foundation: The PBC Foundation, based in the UK, is one of the biggest PBC organizations in the world. Their work is dedicated to providing support and information to those affected by PBC. They offer a variety of patient support services and host events focused around PBC.
New clinical trials are evaluating the safety and efficacy of investigational treatments for PBC and observational monitoring trends in people living with PBC. Interested in finding and participating in a PBC clinical trial near you? Start your search below.