Primary biliary cholangitis (PBC) inflames and damages the small tubes (ducts) in your liver that carry the digestive fluid bile. At first, you might not have any symptoms or problems from the disease. But as bile builds up, it can scar and damage your liver. Getting on an effective treatment can slow the disease’s progression to severe liver scarring, or cirrhosis. It may help delay liver failure and the need for a liver transplant.
Ursodeoxycholic acid (also called ursodiol and UDCA) is often the first medicine doctors prescribe for PBC. UDCA helps clear bile from your liver. This medicine doesn't cure PBC, but it can slow the disease and reduce liver damage. If UDCA doesn't help, you do have other options to manage your condition.
It's important to tell your doctor if your symptoms don't get better so they can adjust your treatment.
Signs That Your PBC Medicine Isn't Working
Up to 40% of people who take UDCA don't improve on it. Their disease may keep getting worse. Around 1 in 10 people have side effects like diarrhea and belly pain that make it hard for them to stay on this medicine.
UDCA may not work as well if you:
- Have cirrhosis
- Have high levels of the liver enzymes gamma-glutamyl transferase and alkaline phosphatase
- Were assigned male at birth
- Were assigned female at birth and were younger than 45 when you were diagnosed with PBC
You may notice symptoms like these if your condition isn't under good control:
- Tiredness
- Itchy skin
- Yellow skin and eyes
- Dry eyes and mouth
- Pain in the upper right part of your belly
- Bone, muscle, or joint pain
- Swelling in your feet, ankles, and belly
Your doctor will do blood tests once every 3 to 6 months, although it takes about a year on treatment to know whether the medicine is working for you. The tests check levels of liver enzymes like alkaline phosphatase and bilirubin in your blood. Higher-than-normal amounts of these substances are a sign that your disease has gotten worse and your liver is damaged. If your liver enzyme levels are high, your doctor might add another medicine to lower them.
Researchers are studying new markers in the genes or blood that might help them predict which people won't respond to treatment or will progress to liver failure more quickly. Anyone who is at risk of progressing could then get more frequent follow-ups and other treatments to prevent their disease from getting worse.
Second-Line Treatments for PBC
If UDCA hasn't helped you or it has stopped working, your doctor might add other medicines or change your treatment. These medications are second-line treatments:
Budesonide. It lowers inflammation, but it can cause side effects like weight gain, itchy skin, and mood changes.
Fibrates. These cholesterol-lowering drugs may help reduce liver inflammation and relieve itching when you take them with UDCA. They work by blocking a type of protein known as a peroxisome proliferator-activated receptor, or PPAR.
Fenofibrate (Tricor). Doctors prescribe this fibrate medication off-label to help with PBC symptoms.We need more research to fully understand how well it works, but it may tamp down liver inflammation and itching when you take it along with UDCA.
Elafibranor (Iqirvo). This is another type of PPAR agonist medication for the treatment of PBC. In clinical trials, it slowed disease progression through a year of treatment and helped to ease PBC-related itching. It eases liver inflammation, cuts down on bile production, and helps your liver move out built-up bile acid.
Seladelpar (Livdelzi). Also a PPAR medication, it helps lower levels of alkaline phosphatase (ALP), an enzyme linked to the progression of PBC. It also helps your liver to make less bile. It can reduce itching in people with PBC whose condition didn't improve enough on UDCA alone.
Obeticholic acid (Ocaliva). This medicine reduces liver inflammation and scarring, and it helps your liver work better. In some people, this medicine can make liver damage worse. If you have cirrhosis, you should not take Ocaliva.
Even though PBC is an autoimmune disease, it doesn't respond well to immune-suppressing medicines like azathioprine (Azasan) and methotrexate (Rheumatrex, Trexall).
Your doctor can also recommend medicines to treat the symptoms of PBC, including:
- Cholestyramine (Locholest, Prevalite, Questran) and antihistamines for itchy skin
- Artificial tears and cyclosporine to relieve dry eyes
- Saliva substitutes to manage dry mouth
- Stimulant drugs like modafinil (Provigil) for tiredness
- Statins to lower cholesterol
- Vitamin D, calcium, and medicines called bisphosphonates to strengthen weak bones
If your disease progresses to liver failure, you may need to get on the list for a liver transplant. A liver transplant may improve your outlook, but your disease could come back years after the surgery.
Self-Care Tips for PBC
Medicine isn't the only way to manage PBC. Try these self-care tips to relieve symptoms and prevent complications of the disease:
- Don't drink alcohol. It damages the liver.
- Take a walk or do other weight-bearing exercises to strengthen your bones.
- Eat more unsaturated fats, like avocado and fatty fish, and fewer saturated fats from red meat and whole-fat dairy products to manage your cholesterol level.
- Avoid raw oysters and other shellfish. They carry germs that might harm your liver.
- Use moisturizers and take baths to relieve itching.
- Tell your doctor before you take any new medicines and supplements. Your liver helps your body break down medicines. Because of liver damage, you could have more side effects when you take certain medicines.
Questions to Ask Your Doctor
To catch problems early, tell your doctor if you have any new symptoms of PBC or side effects from your medication. Use these questions as a guide to talk to your doctor:
- What are my blood test results and what do they mean?
- What symptoms should I watch for?
- Has my disease progressed? What stage is my liver disease?
- How often do I need to have liver function tests?
- Do I need to change or add medicine? Which one do you recommend? What side effects could it cause?
- What lifestyle changes should I make?
Show Sources
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