Primary biliary cholangitis (PBC) is a rare autoimmune disease affecting the bile ducts in the liver. When bile ducts become damaged, bile can build up in the liver, leading to inflammation and scarring.
Bile ducts transport bile to the digestive tract, where they help with digestion. Bile can also help the liver remove toxins.
PBC is a progressive disease, meaning it typically worsens over time. However, someone may not develop symptoms for many years.
Keep reading to learn more about PBC, including its causes, symptoms, and treatment options.
Primary biliary cholangitis (PBC) is an autoimmune disease in which the immune system mistakenly attacks the bile ducts in the liver.
According to the American Liver Foundation, the bile ducts transport bile to the digestive system, where it helps with the digestion of fats and fat-soluble vitamins.
When the ducts become damaged, bile cannot escape the liver. This causes bile to build up, which leads to scarring in the liver.
Eventually, PBC can destroy the bile ducts. As scarring becomes more severe, the liver also becomes less functional.
However, a 2024 article indicates that PBC often progresses slowly over many years, and many people have no symptoms in the early stages. Doctors can prescribe treatments to slow the progression of the disease.
PBC has
- Stage 1 (portal stage): Some inflammation and minor bile duct changes exist.
- Stage 2 (periportal stage): There is some scarring around the portal vein in the liver, which contains the bile ducts. However, the ducts are still intact.
- Stage 3 (septal stage): Further scarring and inflammation occur.
- Stage 4 (biliary cirrhosis): The individual now has cirrhosis, which is severe scarring and liver damage, along with nodules of various sizes.
PBC is an autoimmune disease. In this type of disease, the immune system, which usually protects against infection, attacks healthy tissues instead. In this case, it harms the bile ducts in the liver. Over time, bile accumulates in the liver, causing inflammation, scarring, and cirrhosis.
Doctors do not know why the immune system begins to do this. However, there may be a genetic link, as PBC appears to be more common in some families.
Environmental or other factors may trigger PBC in people with a genetic predisposition to the condition, but further research is necessary to understand how and why PBC develops.
Risk factors
Certain risk factors increase an individual’s chance of developing PBC, including:
- having a parent or sibling with the condition
- being an older adult, as the average age at diagnosis is
60 yearsTrusted Source - being female, as females account for 90% of people with PBC
PBC is also more common in white people than in other ethnicities. It is unclear why the condition affects these groups more than others.
People may experience gradually worsening symptoms as the PBC progresses from early to late stage.
Early stage
Many people have no symptoms in the early stages of PBC, which may continue for years. As a result, doctors diagnose about
However, if someone experiences symptoms, they may include:
- extreme tiredness
- itchy skin
- discomfort in the upper right side of the abdomen
- joint pain or arthritis
Late stage
As the disease progresses, symptoms may include:
- darkening of skin tone
- fatty, yellow bumps on the skin called xanthomas
- symptoms of cirrhosis, such as swelling, jaundice, and weight loss
Advanced PBC may cause certain complications, including:
- Osteoporosis: Osteoporosis develops when bones lose their mineral density, making them weaker and more prone to breaking. There are often no symptoms unless a fracture occurs.
- Malabsorption: Malabsorption occurs when the body does not absorb the vitamins and nutrients it needs from food. It can happen as a result of bile not reaching the digestive tract. People
may haveTrusted Source low levels of vitamins A, D, E, or K. - Steatorrhea: Steatorrhea occurs when the body excretes too much fat in the stool, which may make it paler in color than usual, with an especially unpleasant smell.
- Hyperlipidemia: This is the medical term for high levels of fats in the blood. Hyperlipidemia in itself does not cause symptoms, but it may raise the risk of heart disease. That said,
research has not foundTrusted Source an elevated risk of heart attacks or stroke in those with PBC. - Portal hypertension: Portal hypertension is increased blood pressure in the blood vessels of the abdomen. When this reaches a certain threshold, people can develop swelling in the legs or abdomen, confusion or difficulty thinking, and enlarged veins in the abdomen that could burst if there is enough pressure.
- Liver cancer: People with PBC are more likely to develop a type of liver cancer as a result of cirrhosis.
- Autoimmune thrombocytopenia: This involves a decrease in platelets, which are the cells in the blood that cause clotting. Thrombocytopenia can lead to easy bruising, bleeding of the gums, and internal bleeding.
- Hypoglycemia: Hypoglycemia, or low blood sugar,
can developTrusted Source in those with PBC. - Renal tubular acidosis: Renal tubular acidosis occurs when the kidneys cannot remove acid from the body, leading to a buildup of acid levels.
Doctors diagnose PBC with a blood test. However, because PBC causes no symptoms in the early stages, they may diagnose it during a routine test for something else.
Doctors use blood tests to check:
- Liver enzymes: A high level of alkaline phosphatase (ALP) can indicate PBC.
- Anti-mitochondrial antibody (AMA): This indicator of autoimmunity is present in
85%Trusted Source of people with PBC. - Cholesterol levels: People with PBC may have higher cholesterol levels than those without.
Doctors can diagnose PBC when test results show high levels of ALP and AMA in the blood, even if the person has no symptoms.
To diagnose PBC, doctors might also:
- perform a physical examination, looking at the abdomen and checking for signs of jaundice
- use imaging tests, such as liver ultrasounds, to rule out other causes of the damage
- use liver biopsy to confirm the diagnosis
- take a family history
Doctors may monitor people with PBC by using blood and other screening tests to check vitamin levels and overall liver function.
PBC has no cure, so treatment aims to prevent disease progression and ease symptoms.
Medications
This treatment can help slow the damage and the development of cirrhosis and is most beneficial when people start the drug at an earlier stage of PBC.
Doctors may recommend obeticholic acid (Ocaliva) if someone does not respond to UDCA. However, this option does not improve survival or symptoms.
Surgery
The gold standard of treatment for more advanced PBC is a liver transplant. Therefore, when someone develops cirrhosis complications, doctors should evaluate their suitability for this procedure.
Having a liver transplant involves undergoing a major surgery and taking drugs to prevent the body from rejecting the new organ. These drugs suppress the immune system, which can make a person more vulnerable to infections.
It is possible for PBC to recur after a transplant. Reports suggest recurrence may happen to
Treatment of symptoms and complications
A doctor may prescribe other strategies to manage symptoms or treat the complications of PBC,
- Antihistamines: Antihistamine medications, such as diphenhydramine (Benadryl) or cholestyramine (Questran), may help reduce itchiness.
- Statins: Statins can lower blood fat levels. They are a category of medications that do not harm liver function.
- Nutritional supplements: Supplementation of the fat-soluble vitamins A, D, E, and K may reduce the effects of malabsorption.
- Dietary changes: People with steatorrhea may need to reduce their fat intake. Consuming a generally nutritious and balanced diet
may alsoTrusted Source help preserve liver health as much as possible. Avoiding undercooked meat or fish, shellfish, and unpasteurized milk can also help prevent infections that could be dangerous in those with PBC. - Osteoporosis prevention: This may involve taking oral vitamin D and calcium, getting regular exercise, and, in those experiencing menopause, possible estrogen therapy.
People with PBC must avoid alcohol consumption altogether and stop smoking if relevant as part of their treatment.
There is no cure for PBC. However, the condition can advance slowly, allowing doctors time to provide treatment.
How long can you live with primary biliary cholangitis?
Usually, PBC progresses over the course of 15 to 20 years. Once symptoms develop, the life expectancy is around 10 years, but some people remain symptom-free for many years before this.
The bilirubin level in a person’s blood can be an indicator of the outlook. Bilirubin is a waste product of the breakdown of red blood cells. The liver helps excrete it from the body.
A
How do you prevent primary biliary cholangitis?
Because PBC seems to have a genetic link, people predisposed to it may be unable to prevent it. However, more research into this is necessary.
People may be able to improve their overall liver health by maintaining a moderate weight, eating a balanced diet, exercising, and consuming alcohol responsibly.
People with a family history of the condition can also discuss this with their doctor so they can monitor the situation.
Primary biliary cholangitis (PBC) is a rare autoimmune condition that affects the liver. It damages the bile ducts, causing bile to build up in the liver. Eventually, this leads to scarring and the destruction of the bile ducts.
In the early stages, a person may have no symptoms. However, as the condition progresses and the liver becomes more damaged, they may experience fatigue and symptoms of liver cirrhosis, such as tiredness, jaundice, and itchiness.
Doctors do not know the cause, but genetic factors may be involved. There is no cure, but treatments can help reduce symptoms and slow disease progression.
