Primary biliary cholangitis (PBC) is a liver disease that affects the bile ducts that run through your liver. It slowly degrades those bile ducts, making it harder for bile to flow through. Bile backs up inside your liver, which damages the tissues. Scar tissue gradually replaces healthy tissue and your liver gradually loses its functionality. This is known as cirrhosis. PBC was formerly known as primary biliary cirrhosis.
What are the symptoms of primary biliary cholangitis?
Many people with PBC have no symptoms in the early stages. As the disease progresses, signs of biliary disease begin to appear. The earliest and most common symptoms of people with PBC are:
Fatigue (65%).
Itchy skin (55%).
These symptoms affect different people to different degrees. They can occur later or earlier in the course of your disease, and they can be mild to severe at any stage. How your symptoms present doesn’t seem to be related to how advanced your disease is. However, some research has suggested that more severe symptoms earlier on may predict a faster progression overall.
What are the complications of biliary disease?
You may not notice biliary disease in the early stages. But as it progresses, complications can begin to develop. Bile that can’t flow will begin to leak into your bloodstream, causing illness. Bile that can’t flow also can’t reach your digestive system, where it’s needed to aid digestion. In later stages, scar tissue in your liver begins to affect the blood vessels that pass through it, causing portal hypertension.
Fat malabsorption
When your digestive system lacks the bile it needs, it has trouble breaking down and absorbing fats. Fat malabsorption can cause:
When scar tissue in your liver begins to obstruct the blood vessels that run through it, it causes portal hypertension — high blood pressure in those veins and the veins that branch off from them. Portal hypertension can lead to:
Occasional mental confusion (from the buildup of toxins in your blood).
What treatment is available for primary biliary cholangitis?
Providers use the following treatments for primary biliary cholangitis:
Medication
There’s no cure for PBC, but you can slow it down and improve your condition with medication. Ursodeoxycholic acid (UDCA) is a type of bile salt that can help clear bile from your liver and reduce liver damage. It works well for about half of people with PBC, especially in the early stages. For those who don’t benefit from UDCA, doctors sometimes recommend a different bile salt called obeticholic acid.
Doctors can also treat some of your individual symptoms with different medications. For itching, they may recommend antihistamines such as diphenhydramine (Benadryl® or Aler-Dryl®), ultraviolet light therapy or bile acid sequestrants such as cholestyramine. Vitamin supplements can help prevent vitamin deficiencies and side effects such as osteoporosis. Some people with fatigue benefit from stimulants such as modafinil.
Surgery
If medication doesn’t improve your condition and your liver function continues to decline, your doctor may put you on the liver transplant waiting list. Liver transplant surgery has excellent results for people with PBC. Although, like other autoimmune diseases, PBC may return after your liver transplant, but it tends to progress much more slowly the second time around. Life expectancy after your transplant is normal.
How is primary biliary cholangitis diagnosed?
Your healthcare provider will ask about your medical history and symptoms and physically examine you. Then they’ll test a sample of your blood for evidence of PBC. They look for particular antibodies in your blood that are associated with PBC, especially one called antimitochondrial antibody (AMA). They also look for elevated liver enzymes that indicate liver stress, especially alkaline phosphatase.
If your test results are positive for PBC, your provider will want to look at images of your liver and biliary system next. This helps to rule out other possible causes of your symptoms, and can also help show how advanced the disease is. They’ll usually begin with a simple test like an abdominal ultrasound. But sometimes, they may need to take clearer images with some type of MRI (magnetic resonance imaging).
About 5% of people with PBC test negative for AMA but have other signs and symptoms. In this case, your provider may need to take a liver biopsy to confirm you have PBC. They can usually do this as a bedside procedure using a needle inserted into your liver. The needle will withdraw a tiny tissue sample, and your provider will send the sample to a lab for examination under a microscope.