What is primary biliary cholangitis (PBC)?
Primary biliary cholangitis, formerly known as primary biliary cirrhosis (PBC) is an inflammation of the bile ducts in the liver. This inflammation narrows the ducts and blocks the bile flow. This backing up of the bile (called cholestasis) can gradually damage the ducts and ultimately the liver itself.
What causes PBC?
PBC falls into a broad class of diseases called autoimmune conditions. These can affect different systems in the body, but are thought to be the result of the immune system working against the body itself, in the same way it would work against a foreign object like bacteria or a transplanted organ. The ongoing inflammation causes the tissue damage and resulting symptoms.
What are the symptoms of PBC?
Frequently, there are no symptoms. Most often, people are sent to a gastroenterologist for further evaluation because liver testing shows some abnormality. Fatigue and itching are common. Other possible complications may be bone loss and vitamin deficiencies. Patients with PBC may also have other autoimmune conditions.
How is it diagnosed?
Blood work, specific to the liver, is done. High alkaline phosphotase, antimitochondrial antibody, and antinuclear antibody, are usually seen in PBC. Definitive diagnosis is usually made by liver biopsy. This not only confirms the diagnosis, but allows for assessment of the amount of damage to the liver.
How is it treated?
There is no cure for PBC. You may be treated with a medication called ursodiol. This may slow the progress of the disease if given early in its course. Treatments are directed at controlling the symptoms. For example, medications can be given to decrease itching, or to slow bone loss. Some patients may require vitamin supplements if found to be deficient. To evaluate for bone loss, a bone density (also called DEXA) scan should be done periodically. Some studies indicate that smoking can hasten the progression of the disease, so smoking cessation is very important. Alcohol should also be avoided.
Outlook:
The progression of the disease varies greatly. In some, it is quite slow. In others, it may result in cirrhosis and compromised liver function. Those with advanced cases may be candidates for liver transplant.
When to seek medical advice:
Most patients are sent by their regular doctor for further assessment when liver tests are abnormal. Once a diagnosis is made, there are regular follow up visits. You should also be seen if symptoms such as itching become bothersome.
