Treatment Options for Primary Biliary Cholangitis

By

Erin Azuse

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Your liver plays an important role in digestion and the removal of toxins from within your body. It produces bile, a fluid that passes through small passageways called bile ducts into the small intestine. Bile helps your body break down and absorb essential nutrients while also helping get rid of waste products. However, if you have primary biliary cholangitis (PBC), a disease that damages the bile ducts, this process is affected and can lead to serious health consequences. While there is not currently a cure, there are treatment options available depending upon the stage and severity of the disease.

How does PBC affect your liver?

Primary biliary cholangitis is generally considered an autoimmune disease, meaning your body mistakenly attacks its own tissues. In the case of PBC, this leads to the progressive destruction of the small and medium bile ducts. When bile cannot flow as it should, harmful substances can remain in the liver. This causes inflammation within the liver and can eventually cause cirrhosis, irreversible scarring of the tissues. Increasing damage to the tissues impacts the liver’s ability to function as it should. In advanced cases, PBC can result in liver failure.

What treatments are available for PBC?

Your doctor may prescribe the following medications to slow the progression of PBC:

  • Ursodeoxycholic acid (UCDA): This may also be called ursodiol (Actigall, Urso). UCDA is the most-commonly prescribed first line treatment for PBC. It is a bile acid that naturally forms in the body and helps move bile from the liver to the small intestine. It’s most helpful for those in the early stages of PBC. Weight gain, hair loss, and diarrhea are possible side effects.

  • Obeticholic acid (Ocaliva): This medication decreases the amount of bile that is produced while also increasing its removal from the liver. This may be given as an additional medication if you don’t have a sufficient response to UCDA, or you may take it alone if you aren’t able to tolerate UCDA. Side effects may include itchy skin, fatigue, abdominal and joint pain, and sore throat.

New treatments for PBC are also being investigated. Some examples are:

  • Immunosuppressants: Medications like prednisone, methotrexate (Trexall, Rheumatrex) and colchicine (Colcrys) are often used, but more research is needed to prove their effectiveness.
  • Fibrates: These medications including fenofibrate (Tricor, Triglide) and bezafibrate (Bezalip) have previously been used to treat high cholesterol, but may also be helpful in treating PBC.

When PBC can no longer be controlled with medication, liver failure can occur, and a liver transplant may be needed. Those who receive a liver transplant tend to have good outcomes. However, it is possible PBC can redevelop in the new liver years down the line. Long-term use of immunosuppressive drugs can lower that risk.

Can you treat the symptoms and complications of PBC?

Though they won’t impact the disease itself, talk to your doctor about treatments to help ease symptoms of PBC and decrease the risk of complications:

  • Itching: Many people with PBC have itchy skin. Antihistamines like diphenhydramine (Benadryl) or hydroxyzine (Vistaril) can help. For more severe itching, cholestyramine (Questran) may be recommended. It binds to bile and helps eliminate it through your stool. Rifampin (Rifadin), naltrexone (Vivitorl) and sertraline (Zoloft) may also be prescribed to relieve itching.

  • Dry eyes and mouth: Artificial tear eye drops and saliva substitutes can be purchased without a prescription. If that doesn’t help, your doctor may prescribe pilocarpine (Salagen), cevimeline (Evoxac) or lifitegrast ophthalmic solution (Xiidra).

  • Osteoporosis: Bone loss is a complication of PBC. Vitamin D and calcium supplements may be prescribed.

  • Vitamin deficiencies: The decrease in liver function can lead to a decrease in the absorption of fat-soluble vitamins A, D, E, and K. Supplements of these vitamins may be needed.

To help manage your PBC, it’s important to make healthy lifestyle choices. Don’t smoke or drink alcohol. Exercise daily and drink lots of water. Consider a low sodium diet if you have problems with swelling in your lower extremities or fluid in your abdomen. Don’t take any medications or supplements without first consulting your doctor since many are processed in your liver. Keep in mind that the better you take care of yourself, the better you will feel.

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Medical Reviewers: William C. Lloyd III, MD, FACS Last Review Date: Jan 11, 2018

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View Sources

Medical References

  1. Primary Biliary Cirrhosis. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/primary-biliary-cirrhosis/symptoms-causes/syc-2037687...
  2. Primary Biliary Cholangitis. American Liver Foundation. https://www.liverfoundation.org/for-patients/about-the-liver/diseases-of-the-liver/primary-biliary-c...
  3. Treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis). National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/liver-disease/primary-biliary-cholangitis/treatment

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