The right treatment plan is the best way to manage symptoms of primary biliary cholangitis (PBC). The liver disease, which damages your bile ducts, has no cure, but medication, lifestyle changes, and other treatments can slow it and improve your overall well-being.
How Primary Biliary Cholangitis Affects Your Quality of Life
The symptoms of PBC can affect your quality of life, including everyday tasks. One study looked at how the condition affects people’s lives before it gets severe. Researchers found that people with PBC often have joint pain, which causes them to struggle with chores and other daily tasks and need help more often. People in the study also faced challenges at work and with hobbies and exercise.
The study shows that while your quality of life is generally good with PBC, the illness can still have a big impact on your daily life, relationships, and work.
Primary Biliary Cholangitis Treatment
Treatment for PBC involves medication for the disease, symptoms, and health problems that come with the illness. If you have severe PBC, you may get cirrhosis. In this case, you may eventually need a liver transplant, which may help you live longer.
First-line treatments
Medication can help stop PBC from getting worse and help you avoid further health problems. The first drugs you'll take are:
Ursodeoxycholic acid (UDCA) (Actigall, Urso). UDCA is the go-to medication for treating PBC. It works by improving the flow of bile through your liver. UDCA is not a cure, but it can help slow liver scarring (fibrosis) and improve liver function. Most people respond well to UDCA, and regular blood tests often show improved liver enzyme levels.
However, some people – about 40% – don’t fully respond. And UDCA may not help with symptoms like fatigue or itching, which means some people may need more treatment or second-line therapies to address these gaps.
Second-line treatments
If UDCA doesn’t bring your liver enzyme levels to normal or causes side effects, your doctor may suggest other medications. These can include:
PPAR agonists. These lessen the amount of bile your liver makes. They may improve how well your liver works and can reduce liver injury. You can use them with UDCA, or alone if UDCA doesn't work for you well enough or you can't tolerate it. Some PPAR agonists you may take include:
- Seladelpar (Livdelzi): This newly FDA-approved treatment targets the PPAR-delta pathway, which helps reduce liver inflammation and scarring. It can be used if UDCA doesn’t work well or if you can’t take UDCA. Seladelpar is especially helpful if you have intense itching and fatigue, as it supports better sleep and energy levels.
It also helps to bring down levels of alkaline phosphatase (ALP) in your blood. This is an enzyme that has a link to the progression of liver disease. Side effects are mild and may include nausea and belly discomfort, making it a tolerable and beneficial option for many patients. - Elafibranor (Iqirvo): Elafibranor is another PPAR agonist targeting the PPAR-alpha and PPAR-delta pathways. It’s designed to reduce bile buildup, improve inflammation, and lower liver enzymes such as ALP. It’s usually used with UDCA, especially if you had a poor response to other first-line medications. It also has other benefits, like lowering cholesterol and triglycerides.
Side effects are minimal but can include nausea, diarrhea, and mild stomach pain.
Off-label medicines
Sometimes, medications not officially approved for PBC, known as off-label treatments, can still help you manage symptoms like itching when standard therapies fall short. These may include:
Fibrates (bezafibrate, fenofibrate)
Fibrates are PPAR agonists sometimes prescribed off-label to manage symptoms of PBC when UDCA alone isn’t working. They can decrease bile production and lower triglycerides.
Many patients notice an improvement in itchiness and overall symptoms when they add fibrates to their treatment plan. But you shouldn’t take these drugs if you have severe liver disease or cirrhosis.
What about obeticholic acid (Ocaliva)?
You may be taking obeticholic acid alone or with UDCA. If you take Ocaliva, contact your doctor right away. Recent studies show that obeticholic acid can cause liver injury even if you don’t have cirrhosis.
The manufacturer took Ocaliva off the market and also stopped all clinical trials on the medicine. Your doctor can guide you with the best next steps and help you safely change to another treatment.
When to Consider Second-Line Treatments
Your doctor may recommend second-line treatments if UDCA hasn’t helped your PBC symptoms improve after 6-12 months or if you’re unable to tolerate it.
Other things, like having advanced fibrosis, high liver enzyme levels, or being young at diagnosis, might also make some of these medications a better fit.
PBC Symptom Treatment
PBC symptoms vary but can often be managed with medications, home remedies, and lifestyle adjustments. Common symptoms include fatigue, itching, dry eyes and mouth, and weakened bones.
Fatigue
Fatigue is one of the most common and challenging symptoms of PBC. You can try lifestyle changes, such as improving your diet and staying active, to boost energy levels. Conditions like thyroid problems or anemia, which often come with PBC, can also add to fatigue, so ask your doctor about testing.
For severe fatigue, your doctor might prescribe stimulant medications like modafinil (Provigil).
Itching
Itchy skin (pruritus) can disrupt your sleep and overall comfort. Treatments for itching include:
- Antihistamines. Help reduce itching, especially at night
- Cholestyramine. A powder that binds bile salts in your gut to ease itching
- Rifampin. An antibiotic that blocks itch-inducing brain chemicals
- Opioid antagonists. Drugs like naltrexone that work on your brain to relieve itching
- Sertraline. An antidepressant that may improve itching by boosting serotonin
Second-line PBC treatments like seladelpar and elafibranor can also ease itching.
Dry eyes and mouth
For dry eyes, try artificial tears, which are available over the counter or by prescription. Chewing sugar-free gum or sucking on hard candies can help with a dry mouth. Saliva substitutes are another option.
Bone health (osteoporosis)
PBC can weaken your bones, increasing the risk of fractures. Your doctor may recommend:
- Calcium and vitamin D supplements
- Exercise, particularly weightlifting, to strengthen bones
- Medications like bisphosphonates to prevent bone loss
Lifestyle Changes to Help With Your PBC
Lifestyle adjustments play a major role in managing PBC and protecting your liver health. Try these tips:
Quit smoking. It can worsen liver damage. If you smoke, ask your doctor for help quitting.
Avoid alcohol. Drinking puts extra strain on your liver.
Eat a healthy diet. Limit processed and salty foods. Focus on fresh fruits, vegetables, lean proteins, and whole grains.
Stay active. Exercise, including cardio and strength training, helps with bone health, energy, and overall well-being.
Avoid raw shellfish. They can carry harmful bacteria that are dangerous for people with liver disease.
Be cautious with medicines and supplements. Some can affect your liver. Always check with your doctor before trying anything new.
Practice good dental hygiene. Oral infections can spread and worsen inflammation in your liver.
Stay hydrated. Drinking enough water helps your liver more easily flush toxins from your body.
Manage stress. Chronic stress can impact your liver over time. Try mindfulness techniques, yoga, or talking to a therapist.
Treating PBC Complications
PBC can cause other health problems, such as trouble absorbing vitamins and minerals, high cholesterol, osteoporosis, and more pressure in the blood vessel that carries blood from some organs to your liver (portal vein).
Here are some treatments for these complications:
- Vitamins A, D, E, and K, calcium, folic acid, and iron supplements
- Cholesterol-lowering medication such as a statin
- Reduced-salt foods for elevated pressure in your portal vein (portal hypertension)
Liver Transplant for PBC
If medicine doesn’t control your symptoms and your liver continues to get weaker, you’ll need a liver transplant. This is where a doctor takes out your unhealthy liver and replaces it with a healthy liver from a donor.
Transplants for PBC often have excellent results, and you’ll live a normal life span. But your illness could come back after surgery, even years later. But your symptoms will advance more slowly.
Monitoring Your PBC
Ongoing monitoring is a critical part of PBC treatment. Here’s are some of the tests you can expect from your care team:
- Regular blood tests. These track levels of bilirubin and liver enzymes to see how your treatment is working.
- Imaging tests. Tools like live elastography and magnetic resonance elastography (MRE) detect liver scarring and portal hypertension early.
- Symptom reviews. Regular check-ins ensure your treatment plan is addressing your symptoms effectively.
Future Treatments for PBC
Researchers are working on promising new medications for PBC. These include:
Setanaxib: Setanaxib is a new drug being developed to help with advanced liver scarring in PBC. It works by targeting certain enzymes linked to liver damage. Early studies show it might help lower liver enzyme levels and improve liver health by reducing stiffness.
Although the main goal of its first trial wasn’t fully achieved, other results were encouraging, and more testing is planned. Setanaxib is also being looked at for treating other diseases involving scarring, which could make it a useful option for various conditions.
CNP-104: CNP-104 is a new kind of treatment designed to tackle the autoimmune causes of PBC. It works by training the immune system to stop attacking the liver. The treatment uses tiny particles to deliver a key protein, which helps reduce the harmful immune response.
Early trials showed that CNP-104 can reduce liver damage and improve how the immune system reacts, all without causing serious side effects. If successful, this therapy might remove the need for ongoing medication and provide a long-lasting solution for people with PBC.
With the right combination of medications, healthy habits, and regular monitoring, you can better manage your symptoms, protect your liver, and improve your quality of life.
Show Sources
Photo Credit: DigitalVision/Getty Images
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