How is Primary Biliary Cholangitis treated?
Treating the Disease
There is no cure for PBC, however, there are medications that can help slow disease progression and manage symptoms. Ursodiol (brand names Actigall, URSO 250, URSO Forte) is a naturally occurring bile acid (ursodeoxycholic acid or UDCA) that helps move bile out of the liver and into the small intestine. If used early enough, Ursodiol can improve liver function and may keep you from needing, or delay the need for a liver transplant. People with PBC must take this medication every day for life.
UDCA is effective in more than 50 percent of patients, but up to 40 percent of patients do not achieve an adequate reduction in alkaline phosphotase (ALP) or bilirubin with UDCA, while 5-10 percent are unable to tolerate UDCA.
In May 2016, obeticholic acid (brand name Ocaliva) was approved for the treatment of PBC in combination with UDCA in adults with an inadequate response to UDCA, or as a single therapy in adults unable to tolerate UDCA. Obeticholic acid increases bile flow from the liver and suppresses bile acid production in the liver, thus reducing the exposure of the liver to toxic levels of bile acids. Side effects of obeticholic acid may include increased itching and elevations in blood lipids.
Other alternative therapies in patients who are incomplete responders to UDCA include fenofibrate. Medications to suppress the immune system may also be prescribed including prednisone or azathioprine in PBC patients with the “overlap syndrome” with autoimmune hepatitis. Liver transplantation is considered when medical treatment no longer sufficiently controls the disease. When a person has end-stage liver disease, a liver transplant is necessary for survival.
Treating the Symptoms
Intense itching is one of the most common symptoms of PBC. Over-the-counter antihistamines like diphenhydramine (Benadryl) may be helpful. Other agents such as rifampicin, naltrexone, cholestyramine and sertraline may be prescribed.
Dry eyes can be relieved by using eye drops (artificial tears).
A dry mouth may be helped by sucking on hard candy or chewing gum, both of which increases saliva. There are also saliva substitutes and some medications that can be used.
Blood tests to monitor for deficiencies in fat-soluble vitamins are often done. As PBC progresses, some people need to replace the fat-soluble vitamins lost in fatty stools, so you may be put on vitamin A, D, E and K replacement therapy.
Since people with PBC are at a higher risk for osteoporosis, calcium and vitamin D are usually prescribed. A baseline bone density is currently recommended at the time of initial diagnosis.
As the ability of the liver to function decreases over time, complications associated with cirrhosis will need to be addressed and treated. Screening for varices and liver cancer is often recommended.