What Is Primary Biliary Cholangitis (PBC)?

Primary biliary cholangitis is a rare autoimmune disease that slowly damages the liver's bile ducts. Over time, this can lead to liver damage, which can progress to cirrhosis of the liver. Treatments exist that can slow the progression of the disease and prevent the later stages and complications of PBC.

PBC, formerly known as primary biliary cirrhosis, causes inflammation in the bile ducts. It’s called “primary” because the disease itself is causing the inflammation, rather than its being caused by some other condition, such as an infection or complications from a previous surgery.

The effects of primary biliary cholangitis vary greatly from person to person.

An estimated 50 to 60 percent of people have no symptoms when they are first diagnosed and don’t become symptomatic until the later stages of the disease, while others experience early symptoms that progress quickly.

• Fatigue
• Pruritis, an itchy feeling that makes you want to scratch

Additional symptoms may include:

• Dry skin, dry mouth, or dry eyes
• Abdominal pain
• Patches of darkened skin (hyperpigmentation)
• Jaundice (yellowing of the skin and eyes)
• Joint pain

As the disease progresses, complications can occur. Bile that can’t flow begins to leak into the bloodstream. This not only causes illness, but it also means that there isn’t enough bile to break down and absorb fats.

Fat malabsorption (the inability to absorb fat) can cause the following symptoms:

• Fatty deposits under the skin
• High cholesterol
• Fatty stools or diarrhea
• Weight loss
• Low levels of fat-soluble vitamins A, D, E, and K, especially if your serum bilirubin is above 2 milligams per deciliter (mg/dL).
• Osteoporosis, caused by the failure to absorb fat-soluble vitamin D

Portal hypertension can lead to signs and symptoms including the following:

• Enlarged spleen and liver
• Fluid buildup in the abdomen (ascites)
• Reduced immunity, leading to more colds and infections
• Easy bleeding and bruising (with or without thrombocytopenia, low platelets in the blood)
• Red, spider-like blood vessels under the surface of the skin (spider angiomas)
• Swollen veins in the esophagus and abdomen (varices)
• Gastrointestinal bleeding (from varices or superficial bleeding in the stomach)
• Swelling in the lower body (edema)

• Family History A genetic predisposition increases the likelihood of developing PBC, particularly if a close family member has the disease.
• Environmental Triggers Certain infections, medications (including Tylenol), or toxins may trigger the autoimmune response in those predisposed to PBC.
• Gender The majority of PBC cases occur in women, especially in or after the fourth decade of life, suggesting that hormones might play a role in its development.

Diagnosing primary biliary cholangitis often involves several steps, as there’s no single test to confirm the condition.

A healthcare provider will begin by considering your medical history, family history, physical exam, and a discussion of any symptoms you are experiencing.

Additional diagnostic tests may include the following:

• Blood test that looks for antibodies associated with PBC, particularly antimitochondrial antibody (AMA). The blood will also be tested for elevated liver enzymes that indicate liver stress, especially alkaline phosphatase.
• Imaging tests of your bile ducts, using ultrasound, magnetic resonance imaging (MRI), or both

Although there is no cure for primary biliary cholangitis, there are several treatment options that help slow disease progression as well as manage symptoms and complications.

Medications to Treat Primary Biliary Cholangitis

These drugs work to help slow the progression of primary biliary cholangitis and often help relieve symptoms as well.

Ursodiol (Actigall) is a commonly used medication containing ursodeoxycholic acid (UDCA), which helps protect the liver by reducing the amount of bile acids produced by the liver. It is a first-line treatment for slowing liver damage. The dosage is typically 13 to 15 milligrams per kilogram (mg/kg) of body weight, taken twice daily. UDCA can slow liver disease progression in many patients.

Ursodiol is considered the gold standard of PBC treatment. People who don’t respond to UDCA may be placed on additional medications, typically after one year of treatment.

Obeticholic acid (Ocaliva) is a bile acid modulator that reduces bile acid production and increases bile flow. It's used in people who don’t respond well to UDCA or as an add-on therapy. Dosing starts with 5 milligrams (mg) once daily, increasing to 10 mg depending on tolerance and liver disease stage. The drug may improve liver biochemistry and reduce fibrosis progression.

At the end of 2024, the FDA issued a warning regarding the risk of serious liver injury in patients taking obeticholic acid who did not have cirrhosis of the liver. Such injury had already been seen and warned about in patients with cirrhosis of the liver.

Elafibranor (Iqirvo) is a peroxisome proliferator-activated receptor (PPAR)–alpha and PPAR-delta agonist. It's used alone or in combination with UDCA as an option for second-line PBC therapy in people without decompensated cirrhosis who were intolerant to UDCA or had an inadequate response. The dose is 80 mg once daily. The drug helps control liver inflammation and the levels of certain substances such as bile acids.

Seladelpar (Livdelzi) is a selective peroxisome proliferator-activated receptor-delta (PPARδ) agonist. It's used alone or in combination with UDCA as an option for second-line PBC therapy in people without decompensated cirrhosis who were intolerant to UDCA or had an inadequate response. The dosage is 10 mg once daily. The drug helps lower levels of alkaline phosphatase (ALP), a liver enzyme and key indicator of PBC, and helps relieve itching.

Fibrates (fenofibrate, bezafibrate)
modulate bile acid metabolism and have anti-inflammatory effects.
They're used off label in patients with incomplete response to UDCA. Fibrates are not used in people with decompensated cirrhosis. Fibrates may improve liver function tests and itching.

Symptom Management for Primary Biliary Cholangitis

These medications can help relieve symptoms, especially itching. Most are not specifically approved to treat PBC but rather are used off label.

Antihistamines, including over-the-counter antihistamines such as diphenhydramine (Benadryl) and loratadine (Alavert, Claritin), may help with itching.

Medical Procedures

Immunizations

Supportive Medications

Vitamin and mineral supplements
are used to address deficiencies due to malabsorption (fat-soluble vitamins A, D, E, K, and calcium), especially if serum bilirubin is greater than 2mg/dL. They can also prevent complications like osteoporosis.

Surgery: Liver Transplantation


The cause of primary biliary cholangitis isn’t completely understood.

While lifestyle changes won’t treat the disease directly, they can help to slow its progression, limit or prevent complications, and improve quality of life.

• Limit or avoid alcohol. People with PBC should stop drinking alcohol (especially heavy alcohol use).
• Stop smoking.
• Be physically active. Regular exercise is recommended. Because people with PBC are at higher risk for developing osteoporosis, activities such as walking and resistance training are especially important. Physical activity may also help reduce fatigue by improving blood circulation.
• Eat a healthy diet. Eat a healthy and well-balanced diet. People with edema or ascites should eat a low-sodium diet, and people who have nonalcoholic steatohepatitis also known as fatty liver should reduce fat intake.
• Avoid certain foods. Avoid eating raw or undercooked shellfish, meat, and unpasteurized milk, because these could cause severe infection.

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• Reduce your fall risk. Minimize your risk for falls by clearing floors and walkways of clutter and tripping hazards. Add grab bars and secure mats in bathrooms, tubs, and showers, and make sure all areas in your home are well lit.
• Get regular checkups. See a liver specialist regularly to manage your medications and address any health issues you may have.

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Once a person has PBC, it takes about 15 to 20 years for it to progress to end stage liver disease (when it can’t be cured, and death is the expected result). Once people have symptoms, the average life expectancy is about 10 years.

As liver damage worsens, primary biliary cholangitis can cause serious health problems, including:

Cirrhosis Cirrhosis is scarring of the liver, which lowers liver function and may lead to liver failure. It means the later stage of primary biliary cholangitis. People with both PRC and cirrhosis have a poor prognosis and a higher risk of other complications.

Portal Hypertension Portal hypertension is high blood pressure in the portal vein. The portal vein is located in your belly (abdomen). It gets blood from your digestive organs (large and small intestines, stomach, pancreas, spleen) and carries it to the liver.

Esophageal Varices Enlarged veins in the esophagus, the tube that joins the stomach and throat, are known as esophageal varices. Portal hypertension is a potential outcome of cirrhosis in PBC, and esophageal varices are a severe consequence of portal hypertension.

Splenomegaly The spleen may become swollen with white blood cells and platelets because the body no longer filters toxins out of the bloodstream as it should.

Gallstones and Bile Duct Stones If bile cannot flow through the bile ducts, it may harden into stones in the ducts.

Liver Cancer Liver scarring, or cirrhosis, increases your risk of liver cancer.

Osteoporosis PBC increases the risk of weak, brittle bones that may break more easily.

Vitamin Deficiencies Not having enough bile affects the digestive system's ability to absorb fats and the fat-soluble vitamins, A, D, E, and K, which may cause vitamin deficiencies. Low levels can result in a variety of health problems, including night blindness and bleeding disorders.

High Cholesterol Up to 80 percent of people with primary biliary cholangitis have high cholesterol.

Primary biliary cholangitis affects an estimated 30,000 to 50,000 people in the United States, with women making up about 75 to 80 percent of cases. The disease most commonly occurs in individuals aged 40 to 60, though it can develop at any age.

The disease is notably more severe in some minority groups, with disparities in diagnosis and treatment contributing to poorer outcomes for Black and Hispanic patients.

Although more research is needed to determine all the factors contributing to disparities, Black patients with PBC often don't receive UDCA, which is key for survival.

Life With PBC: An American Liver Foundation Support Group

This private American Liver Foundation Facebook community is open only to people with primary biliary cholangitis. It provides information and answers questions posed by members.

PCBers Organization

A nonprofit, volunteer-run organization, PCBers Organization offers support and education to those affected by PCB and raises money for PCB research.

• Primary biliary cholangitis is an autoimmune disease that damages the liver's bile ducts, potentially leading to serious complications like cirrhosis and liver failure.
• While there is no cure for PBC, medications like ursodiol can help slow disease progression, and additional treatments are available for symptom management.
• Consistent medical management is crucial in slowing liver damage and improving quality of life, so seeking medical advice if you have any symptoms of PBC and getting regular checkups if you’ve been diagnosed with it is recommended.
• It's important to manage lifestyle factors, like alcohol and smoking, to mitigate the disease's progression and to maintain healthy habits, like exercising and following a healthy diet.