Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease that harms the liver’s ability to function. It is chronic, which means it lasts for a long time or regularly comes back.
In people with PBC, the bile ducts become injured, then inflamed, and eventually permanently damaged. The bile ducts are small tubes in the liver that carry bile (a substance needed to digest food) from the liver to other parts of the digestive system.
When the bile ducts don’t work, bile builds up in the liver. This buildup can cause scarring in the liver (cirrhosis). Cirrhosis makes it hard for the liver to function properly. PBC is progressive, meaning it gets worse over time. If it is not treated, cirrhosis can cause liver failure and even death.
Doctors typically diagnose PBC in people between 35 and 60 years of age. Over 90 percent of patients who develop PBC are women. It affects people of all races. PBC is estimated to occur in about 65 of every 100,000 women and in 12 of every 100,000 men.
Doctors do not know for certain what causes PBC. Some experts believe it may be related to a problem with the immune system, where the immune system attacks healthy cells in the body. In PBC, doctors believe the immune system damages the bile ducts.
While there is not a known cause, some people with PBC also have other related autoimmune diseases. These conditions include autoimmune hepatitis, thyroid diseases, scleroderma, Raynaud’s diseases, Sjogren’s syndrome and celiac disease. Frequent urinary tract infections are also often seen in patients with PBC. In addition, infections, smoking and exposure to certain chemicals may play a role in triggering PBC.
PBC also tends to occur more commonly among family members. If one member of a family has PBC, the other family members are at an increased risk.
Many people with PBC have no symptoms in the early stages. Some people discover they have the condition when a doctor tests them for another issue. As the condition progresses, affected people may first experience symptoms including:
In later stages of PBC, additional symptoms include:
To diagnose PBC, a doctor will ask about your medical and family history, do a physical exam, order blood tests and other medical tests. Doctors use a blood test to look for a specific substance in the blood called anti-mitochondrial antibody (AMA). The presence of this substance almost always confirms PBC. Other blood test results in patients with PBC may reveal higher than normal levels of liver enzymes and higher levels of cholesterol and triglycerides. Blood work, specific for liver disease, will also be checked.
If you do have PBC, your doctor may also use an ultrasound test, which provides images of the liver and the bile ducts. Ultrasound enables doctors to see any other abnormal findings in the liver. Sometimes, a doctor will perform a liver biopsy to confirm the diagnosis. In this test, your doctor removes a small piece of the liver using a small needle and looks at it under a microscope.
There is no known cure for PBC. Doctors use medication to manage symptoms and slow the progression of PBC. Ursodiol (Actigall®) helps remove bile from the liver. This treatment improves liver function and prevents progression of the liver disease in many cases. Another drug, obeticholic acid (Ocaliva®) is used in combination with ursodeoxycholic acid (UDCA) or alone if UDCA is not effective or cannot be tolerated. This therapy reduces the amount of bile produced and increases the flow of bile out of the liver.
Other drug options are available depending on each patient’s unique situation and presence of other medical conditions. A liver transplant may be needed if liver function continues to worsen despite medical treatment.
To treat the common problem of intense itching, diphenhydramine (Benadryl®; available over-the-counter) or the prescription drugs hydroxyzine (Vistaril®) or cholestyramine (Locholest®, Questran®) may be prescribed.
Some people with PBC develop osteoporosis (thinning of the bones), which can lead to bone breaks (bone fractures). This complication typically occurs when a person is in the later stages of the disease. Doctors often treat osteoporosis by recommending regular exercise and prescribing medicines such as alendronate (Fosamax®) or risedronic acid (Actonel®). Both of these treatments help strengthen bones.
Other complications include high cholesterol levels, fat soluble vitamin deficiency, and an increased risk of liver cancer.
Because doctors do not know the cause of PBC, it cannot be prevented. However, you can take steps to lessen liver damage, including:
Healthy food choices could include eating foods rich in vitamins A, D, E and K and/or supplements of these vitamins. Also, foods high in vitamin D and calcium could help prevent osteoporosis. Ask your doctor for specific foods high in these vitamins and minerals.
Foods to avoid include raw shellfish (because of possible bacteria presence); plus food high in salt, fat, and carbohydrates – especially added sugars.
In most cases, PBC progresses slowly. Early diagnosis and treatment are important to managing PBC symptoms and avoiding progression of the disease. For many people with the disease, medications and lifestyle changes can control symptoms, so the condition does not interfere with daily life.
If PBC is not treated, and liver function gets worse, it can lead to more serious health problems. People with PBC that progresses to the late stages may need a liver transplant. The prognosis for these people after a transplant is excellent.
Call your doctor if you develop symptoms of PBC. It is important to get an accurate diagnosis of liver problems so that you can treat them as soon as possible.
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This information is provided by the Cleveland Clinic and is not intended to replace the medical advice of your doctor or healthcare provider. Please consult your healthcare provider for advice about a specific medical condition. This document was last reviewed on: 02/21/2018