Primary biliary cholangitis

Overview

What is Primary Biliary Cholangitis?

Primary Biliary Cholangitis (PBC) is a chronic liver disease resulting from the progressive destruction of the small bile ducts. It is an autoimmune disease in which a person’s own immune system mistakenly attacks the healthy bile duct cells. As the bile ducts become injured, inflamed, and eventually destroyed, bile builds up and causes liver damage. This can eventually lead to permanent scarring of the liver. As the scar tissue replaces the healthy tissue, liver function becomes impaired.

How common is Primary Biliary Cholangitis?

Approximately 60 out of 100,000 U.S. women and 15 out of 100,000 U.S. men have PBC. PBC is usually diagnosed between ages 35 to 60 years. It appears to be more common among siblings and family members, suggesting a genetic component.

1, 2

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1
Primary Biliary Cholangitis (PBC). American Liver Foundation. Published March 22, 2024. Accessed Jul 15, 2024. liverfoundation.org-pbc
2
Primary Biliary Cholangitis (Primary Biliary Cirrhosis). National Institute of Diabetes and Digestive and Kidney Diseases. Published March 2021. Accessed Jul 15, 2024. www.niddk.nih.gov-pbc

Symptoms

What are the symptoms of Primary Biliary Cholangitis?

Many people do not present with any symptoms when first diagnosed. PBC is typically suspected due to abnormal blood tests.

The most common symptoms of PBC include:

Feeling tired
Itchy skin
Discomfort or pain in the right, upper side of abdomen
Joint pain
Dry eyes
Dry mouth

As the disease progresses the following symptoms may be observed:

Darkening of skin color
Yellow bumps on skin of fatty deposits called xanthomas
Symptoms of cirrhosis like edema, jaundice, and weight loss

How is it diagnosed?

PBC is typically diagnosed by evaluating medical and family history, a physical exam, and the results of bloodwork tests. Providers might inquire whether there is a history of certain autoimmune diseases, if parents or siblings have been diagnosed with PBC, or if there is a history of chemical exposure or infections.

A physical exam may involve an examination of your abdomen by pressing on specific abdomen regions and/or listening to sounds in the abdomen with a stethoscope. Providers will also look for yellowing of the whites in the eyes and skin, checking if the spleen and liver are of abnormal size, and checking for abdominal tenderness, specifically in the right, upper side of the abdomen.

Because many people with PBC present with no initial symptoms, routine blood work can help identify PBC. Abnormal liver enzyme levels can be a sign of liver damage. Elevated alkaline phosphatase (ALP) levels and the presence of antimitochondrial antibodies (AMA) in the blood are strong indicators of a PBC diagnosis. Cholesterol levels higher than normal are also seen in people with PBC.

Additionally, a liver biopsy can be done to confirm a PBC diagnosis or determine how advanced the disease is. Imaging test may be used to rule out other diseases.

Medications

The following specialty medications are available at Accredo, a specialty pharmacy for Primary Biliary Cholangitis

Medication Medication	Manufacturer Manufacturer
Medication IQIRVO® (elafibranor)	Manufacturer Ipsen Biopharmaceuticals, Inc.
Medication Ocaliva™ (obeticholic acid)	Manufacturer Intercept Pharmacueticals, Inc.

Financing Your Care

Financial resources are available to support your drug costs, including manufacturer and community programs. You may also use the copay assistance search for additional programs. Accredo specialists are available to help you find the right program for you.

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