Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a disease caused by damage to bile ducts in the liver. These small channels carry the digestive fluid, or bile, from the liver to the small intestine.
In the intestine, bile helps break down fat and aids in the absorption of fat-soluble vitamins, such as A, D, E, and K.
Damage to the bile ducts allows bile to build up in the liver. Over time, the accumulated bile damages the liver. It can lead to permanent scarring and cirrhosis.
After their diagnosis, a person doesn’t typically experience symptoms until 2 to 4.2 years later. However, some people may not have symptoms for over 17 years. And if a person has an earlier stage of PBC (stage 1 or 2), their life expectancy is average.
However, everyone is different. Some people live longer than others with the disease. New treatments are improving the outlook for people with PBC.
PBC has four stages. They’re based on how much damage has been done to the liver.
- Stage 1. There’s inflammation and damage to the walls of medium-sized bile ducts.
- Stage 2. There’s blockage of the small bile ducts.
- Stage 3. This stage marks the beginning of scarring.
- Stage 4. Cirrhosis has developed. This is permanent, severe scarring and damage to the liver.
PBC develops slowly. You may not have any symptoms for years, even after you’ve received a diagnosis.
The first symptoms are often fatigue, dry mouth, and dry eyes along with itchy skin.
Later symptoms can include:
- belly pain
- darkening of the skin
- appetite loss
- weight loss
- dry eyes and mouth
- small yellow or white bumps under the skin (xanthomas) or eyes (xanthelasmas)
- joint, muscle, or bone pain
- yellowing of the skin and eyes (jaundice)
- swollen belly from fluid buildup
- swelling in the legs and ankles (edema)
- fractures caused by weakened bones
Possible complications of PBC depend on the stage you’re in, and can vary from person to person. They may include:
Progressive liver damage (cirrhosis)
Not only does cirrhosis affect the liver’s ability to perform its tasks, but it also increases the risks for liver cancer.
Patients with cirrhosis can also experience portal hypertension. This condition causes high pressure in the portal vein that leads to the liver. Portal hypertension can cause serious complications that include:
- ascites — fluid buildup in the abdomen
- enlarged spleen (splenomegaly)
- low platelet levels, which can lead to bleeding
- varices — enlarged veins in the gastrointestinal tract
A major problem with PBC is cholestasis — when bile flow stops — within the liver, which causes the liver problems of PBC. However, cholestasis also leads to other problems when bile doesn’t go to other parts of the body that need it to function properly, like the intestine and gallbladder.
Enlarged spleen (splenomegaly)
As liver cirrhosis caused by PBC progresses, it can lead to an enlarged spleen.
About 30% of people with PBC experience mild changes in bone density known as osteopenia. Another 10% experience significant bone density losses enough to cause osteoporosis. It’s likely that people with PBC experience bone changes because cholestasis associated with PBC means there’s less bile in the intestines. This lack of bile in the intestines contributes to vitamin deficiency (A, D, E, K) in your body, since bile helps your body absorb these vitamins. One consequence is reduced bone health because vitamin D deficiency is related to low bone density.
Increases in blood lipids
An estimated 85% of PBC patients experience high levels of serum cholesterol and triglycerides. However, doctors haven’t linked these higher levels with increased risks for heart-related complications.
People who have cirrhosis are about
In jaundice, bile from the liver does not flow through the typical channels out of the liver and into the intestine. Instead, bile seeps into the bloodstream in higher than average amounts, causing a yellow hue in the eyes and skin.
PBC is an autoimmune disease. This means your immune system mistakes tissue in your liver for a foreign invader and attacks it.
Your immune system has an army of “killer” T cells that identify and fight off harmful invaders like bacteria and viruses. In people with PBC, these T cells mistakenly attack the liver and damage cells in the bile ducts.
Doctors don’t know exactly what causes this immune system attack. It’s likely triggered by both genetic and environmental causes.
Additional risk factors include:
- being between the ages of 30 and 60
- having a parent or sibling with this condition
- smoking cigarettes
- being exposed to certain chemicals
PBC is more common in some areas of the world than in others. The highest rates of the condition are in the United States, where there are 402 cases per million residents. The next-highest location is England, with 240 cases per million residents, followed by Greece. Countries such as Australia and Canada have some of the lowest rates of PBC.
Women also experience PBC at higher rates, sometimes as high as 10 times compared to men.
Although there’s no cure for PBC, treatments can improve your symptoms and protect your liver from further damage. Your treatment may also depend on the complications your PBC is causing. Treatment options include:
The first treatment doctors usually try is ursodeoxycholic acid (UDCA) or ursodiol (Actigall, Urso).
Ursodiol is a bile acid that helps move bile from the liver into the small intestine. It can help slow liver damage, especially if you start taking it when the disease is still in the early stages.
You’ll need to keep taking this drug for life. Side effects of ursodiol include weight gain, diarrhea, and hair loss.
Obeticholic acid (Ocaliva) is a newer drug that’s been approved for use in people who either can’t tolerate UDCA or who don’t respond to it. This drug lowers the amount of bile in the liver by reducing bile production and helping push bile out of the liver.
Some doctors prescribe immune-suppressing drugs to prevent the immune system from attacking the liver. These drugs include methotrexate (Rheumatrex, Trexall) and colchicine (Colcrys). However, they haven’t been proven effective for PBC specifically.
Treatments for complications
Your doctor can also prescribe medications to treat symptoms and complications of PBC, such as:
- for itching: antihistamines like diphenhydramine (Benadryl), hydroxyzine (Vistaril), or cholestyramine (Questran)
- for dry eyes: artificial tears
- for dry mouth: saliva substitutes
- for high cholesterol: fibrates, such as clofibrates, gemfibrozil, and fenofibrate
You’ll also need to avoid alcohol since it can further damage your liver.
If you become deficient in fat-soluble vitamins, you can take supplements to replace them. Taking calcium and vitamin D can help keep your bones strong.
Because PBC causes no symptoms in its early stages, it may be diagnosed during a routine blood test that your doctor orders for another reason.
Your primary care doctor or a liver specialist — called a hepatologist — can diagnose PBC. The doctor will first ask about your symptoms, health history, and family medical history. You’ll also have a physical exam.
Tests used to diagnose this condition include:
- blood tests: Your doctor may order blood tests to check liver enzymes and other measures of liver function.
- antimitochondrial antibody test (AMA): Your doctor may order an antimitochondrial antibody test (AMA) to check for autoimmune disease.
- liver biopsy: Your doctor may order or perform a liver biopsy, which removes a small piece of the liver for examination.
Your doctor may also order imaging tests to make a diagnosis. These include:
PBC is chronic and progressive. It’s not curable, and over time it can lead to permanent liver damage.
PBC often develops slowly, however. This means you may be able to remain healthy for many years with few symptoms. Once you develop symptoms, medication may help manage them.
Better treatments have improved the outlook for people with PBC in recent years. People who respond to treatment earlier in the course of the illness are more likely to have a typical life expectancy. The American Liver Foundation states that ursodiol works in about 50% of the people who take it. For the rest, liver damage may continue.
If your liver becomes too damaged to work properly, you’ll need a liver transplant. This surgery replaces your liver with a healthy one from a donor.
To have the best possible outlook, follow the treatment your doctor prescribes. Stay healthy with diet, exercise, and not smoking or drinking alcohol.