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 vitamins.

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.

Everyone is different. Some people live longer than others. New treatments are helping people with the disease.

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.

The PBC develops slowly. You may not have any symptoms for a long time after you get a diagnosis.

The first symptoms are often fatigue, dry mouth, and dry eyes along with itchy skin.

Symptoms that can include later can be found.

  • There is belly pain.
  • The skin is dark.
  • nausea
  • 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)
  • The belly is swollen from fluid.
  • swelling in the legs and ankles (edema)
  • There is a lot of diarrhea.
  • Weak bones cause the broken bones.

There are possible consequences of the stage you are in. 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.

Portal hypertension

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:

Cholestasis

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)

An enlarged spleen can be caused by the progression of liver cirrhosis.

Bone disease

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.

Autoimmune disorders

As many as 70% of patients with PBC will also have another autoimmune disease. These include conditions such as Raynaud’s and keratoconjunctivitis sicca (dry eye).

Gallstones

People who have cirrhosis are about twice as likely to have gallstones. Cirrhosis of the liver affects how the liver processes bile, making it more likely to form gallstones.

Jaundice

bile from the liver does not travel through the normal channels into the intestine in jaundice. bile enters 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 a lot of T cells that fight off harmful invaders. T cells mistakenly attack the bile ducts in people with PBC.

Doctors don’t know exactly what causes this immune system attack. It’s likely triggered by both genetic and environmental causes.

Doctors have identified certain genes that seem to make a person more likely to develop PBC. When people with these genes are exposed to certain environmental factors, an autoimmune reaction that causes PBC can occur.

Additional risk factors include:

  • Being between 30 and 60.
  • A parent or sibling has this condition.
  • smoking
  • Being exposed to something.

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.

Treatments can help you with your symptoms and protect your liver from further damage. Your treatment may be affected by the problems your PBC is causing. Treatment options include:

Ursodeoxycholic acid

URSA or URSodiol is the first treatment doctors usually try.

bile from the liver is moved into the small intestine by uradiol. It can help slow the damage to the liver if you start taking it.

You’ll need to keep taking this drug for life. Side effects of ursodiol include weight gain, There is a lot of diarrhea., and hair loss.

Obeticholic acid

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.

Immunosuppressants

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 prescribe drugs to treat symptoms of PBC.

  • 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

Alcohol can damage your liver.

If you become deficient in fat-soluble vitamins, you can take supplements to replace them. Taking calcium and vitamins D and K can help your bones.

Because there are no symptoms in the early stages of PBC, it may be diagnosed during a blood test that your doctor orders.

A hepatologist can diagnose PBC. The doctor will ask about your symptoms, health history and family history. You will have a physical exam.

This condition can be diagnosed with tests used.

  • 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 order tests to make a diagnosis. These include:

PBC is progressive and chronic. It can lead to permanent damage to the liver.

The PBC develops slowly. You may be able to remain healthy for many years with few symptoms. If you develop symptoms, medication may help.

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.

“Follow the treatment your doctor prescribes to have the best outlook. Don’t smoke, drink or eat alcohol, and stay healthy.”