Because no cure exists for primary biliary cirrhosis, treatment focuses on slowing the progress of the disease, relieving symptoms and preventing complications.
Treating the disease
Treatments aimed at slowing the disease and prolonging life include:
Ursodeoxycholic acid (UDCA). Also known as ursodiol (Actigall, Urso), UDCA is a bile acid that helps move bile through your liver. UDCA doesn't cure primary biliary cirrhosis, but it may prolong life if started early in the disease and is commonly considered the first line of therapy.
Although UDCA doesn't work for everyone with primary biliary cirrhosis, people with early-stage disease respond better. UDCA is less likely to help people with advanced liver damage. Side effects may include weight gain, hair loss and diarrhea.
Liver transplant. When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant is a procedure to remove your diseased liver and replace it with a healthy liver from a donor. Most donated livers come from people who have died. But in some cases, it may be possible to remove a portion of a liver from a living donor.
Primary biliary cirrhosis often recurs in the transplanted liver, but it may take several years to develop. People with primary biliary cirrhosis who have liver transplants generally have high survival rates — 80 to 85 percent after five years on average.
New medications. Researchers continue to explore other drugs for treating primary biliary cirrhosis. Immunosuppressant drugs, in particular methotrexate (Trexall, Rheumatrex) and colchicine (Colcrys), have been widely used, but their effectiveness remains unproved.
Numerous other drugs, including antiviral medications, continue to be studied as well. Studies suggest that adding fenofibrate (Tricor, Triglide, others) or bezafibrate (Bezalip, others) may be helpful for some people who don't respond to UDCA alone, but larger studies are needed.
Treating the symptoms
Your doctor may recommend treatments to control the signs and symptoms of primary biliary cirrhosis to make you more comfortable. Treatments may help control the most common symptoms.
Treatment for fatigue
Although primary biliary cirrhosis itself causes fatigue, your daily habits or and other medical conditions can contribute to your tiredness. By addressing these other factors, you may get reliev. One medication that has shown promise in studies is modafinil (Provigil). More research is needed to determine its role in primary biliary cirrhosis.
Treatment for itching
- Antihistamines such as diphenhydramine (Benadryl, others) and loratadine (Alavert, Claritin, others) are commonly used to reduce itching from insect bites dry eyes and dry mouth. On the other hand, antihistamines can help with sleep if itching keeps you awake.
- Cholestyramine (Questran, Prevalite) is a powder that must be mixed with food or liquids. Though cholestyramine works for most people, the taste is unpleasant.
- Rifampin (Rifadin, Rimactane, others) is an antibacterial drug which is taken in pill form. Exactly how rifampin reduces itching is unknown, but it may block the brain's response to itch-inducing chemicals in your circulation.
- Opioid antagonists such as naloxone (Bunavail, Evzio) and naltrexone (Vivitrol) may help itching related to liver disease. Like rifampin, these drugs seem to reduce the itching sensation by acting on your brain.
Treatment for dry eyes and mouth:
- Artificial tears and saliva substitutes are available over-the-counter.
- Pilocarpine (Isopto Carpine, Salagen) is a prescription medicine that may be used if over-the-counter medications don't help.
- Cevimeline (Evoxac) is another prescription medicine for dry eyes and mouth.
Working together, you and your doctor can help prevent some of the complications that can occur with primary biliary cirrhosis:
May 16, 2017
- Increased pressure in the portal vein (portal hypertension). Your doctor is likely to screen for portal hypertension and enlarged veins when you're first diagnosed and every few years thereafter. If you're diagnosed with portal hypertension or bleeding, treatment may involve medications, such as a beta blocker or nitrate, or surgery.
- Weak bones (osteoporosis). Treatment for bone loss associated with primary biliary cirrhosis often involves calcium and vitamin D supplements. Your doctor may also recommend that you exercise most days of the week to help increase your bone density.
- Vitamin deficiencies. Your doctor may recommend supplements of vitamins A, D, E and K to counter vitamin deficiencies. Avoid taking herbs or nutritional supplements without talking to your doctor first.
- Ferri FF. Ferri's Clinical Advisor 2014: 5 Books in 1. Philadelphia, Pa.: Mosby Elsevier; 2014. https://www.clinicalkey.com. Accessed Oct.8, 2014.
- Picco MF (expert opinion). Mayo Clinic, Jacksonville, Fla. Oct. 25, 2014.
- Primary biliary cirrhosis. The National Digestive Diseases Information Clearinghouse. http://digestive.niddk.nih.gov/ddiseases/pubs/primarybiliarycirrhosis/index.aspx. Accessed Oct. 8, 2014.
- Juran BD, et al. Environmental factors in primary biliary cirrhosis. Seminars in Liver Disease. 2014;34:265.
- Lindor, KD, et al. Primary biliary cirrhosis. American Association for the Study of Liver Diseases Practice Guidelines. Hepatology. 2009;50:291.
- Poupon R. Clinical manifestations, diagnosis, and natural history of primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Boyer TD, et al., eds. Zakim & Boyer's Hepatology: A Textbook of Liver Disease. 6th ed. https://www.clinicalkey.com. Accessed Oct. 8, 2014.
- Poupon R. Overview of the treatment of primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Flamm S, et al. Liver transplantation in primary biliary cirrhosis. http://www.uptodate.com/home. Accessed Oct. 8, 2014.
- Honda A, et al. Anticholestatic effects of bezafibrate in patients with primary biliary cirrhosis treated with ursodeoxycholic acid. Hepatology. 2013;57:1931.
- AskMayoExpert. What is magnetic resonance elastography (MRE) and what are the indications for an MRE examination? Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Singal AK, et al. Model for end-stage liver disease. Journal of Clinical and Experimental Hepatology. 2013;3:50.
- PCBers. http://pbcers.org/. Accessed Oct. 8, 2014.
- Mells GF, et al. Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study. Hepatology. 2013;58:273.
- American Liver Foundation. http://www.liverfoundation.org/support/. Accessed Oct. 21, 2014.
- Venkatesh SK, et al. Magnetic resonance elastography of liver: Clinical applications. Journal of Computer Assisted Tomography. 2013;37:887.
- Imam MH, et al. Pathogenesis and management of pruritus in cholestatic liver disease. Journal of Gastroenterology and Hepatology. 2012;27:1150.
- Bunchorntavakul C, et al. Pruritis in chronic liver disease. Clinics in Liver Disease. 2012;16:331.
- Golden AK. Decision Support System. Mayo Clinic, Rochester, Minn. Oct. 6, 2014.
- Martin P, et al. Evaluation for liver transplantation in adults: 2013 Practice guideline by the American Association for the Study of Liver Diseases and the American Society of Transplantation. Hepatology. 2014;59:1144.