Overview

Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is a chronic liver disease resulting from progressive destruction of the bile ducts in the liver – called the intrahepatic bile ducts. Bile produced in your liver travels via these ducts to your small intestine where it aids in the digestion of fat and fat-soluble vitamins (A, D, E and K). When the ducts are destroyed, bile builds up in the liver contributing to inflammation and scarring (fibrosis). Eventually this can lead to cirrhosis and its associated complications, as scar tissue replaces healthy liver tissue and liver function becomes increasingly impaired.

Causes

The causes of primary biliary cholangitis (PBC) remain unknown. But current evidence suggests that PBC occurs in people with a genetic predisposition, however, there may also be other factors that combine with genetics to trigger the disease. Medical scientists believe that PBC is an autoimmune disease. This means that your immune system, which protects against infections and cancer, mistakes cells in the bile ducts as being abnormal and attacks them. Some factors—such as an infection or some form of toxic exposure from the environment—may trigger your immune system into making this mistake. PBC usually develops and progresses slowly, which means that liver damage typically gets worse over a long period of time. Medication can slow this progression, especially if you get treated early. It’s important to note that PBC is not caused by alcohol consumption.

Symptoms

The most common symptoms of PBC are fatigue ranging from mild to disabling, and itching in any part of the body. Itching may also range from mild to intense, often occurring on palms or soles of the feet and may be the result of the liver’s inability to process bile. Other PBC symptoms may include gradual darkening of the skin and small yellow raised areas under the skin (usually at the inside corners of the eyes). Many people with PBC never develop any symptoms related to the disease.

Diagnosis

PBC is usually diagnosed with the following blood tests:

Antimitochondrial Antibody (AMA)

Alkaline Phosphatase (ALP or Alk-Phos)

Alanine Aminotransferase (ALT) and Aspartate Aminotransferase (AST).Immunoglobulin (IgM)

Abdominal ultrasound.

Liver biopsy

Treatments

PBC is a chronic liver disease that can be controlled in most people but not cured. Medications such as ursodeoxycholic acid (also called UDCA or URSO) can improve liver function and slow the onset of fibrosis (scar tissue) in the liver. Obeticholic acid (OCA), a manufactured bile acid that helps reduce liver inflammation and cholestasis (restricted bile flow), is available in Canada.