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Understanding Primary Biliary Cholangitis (PBC): Symptoms, Causes, and Treatment Options

Jerome Albert Ecker | MD

Medically reviewed by Jerome Albert Ecker | MD, Assistant Professor of Medicine, Duke University - Durham, NC on January 18th, 2024.

Key Takeaways

  • This autoimmune condition destroys small bile ducts in the liver, causing bile acids to accumulate and trigger inflammation that can progress to cirrhosis over 10-20 years without treatment.
  • Women account for 90% of cases, typically diagnosed between ages 40-60, with strongest risk factors being family history and concurrent autoimmune diseases like Sjögren's syndrome or thyroid disorders.
  • The hallmark symptom is severe pruritus (itching) affecting 80% of patients, often starting on palms and soles before becoming generalized—this can occur years before other symptoms appear.
  • Ursodeoxycholic acid (UDCA) 13-15 mg/kg daily is first-line treatment and can normalize liver enzymes in 60% of patients when started early, significantly improving transplant-free survival.
  • Elevated alkaline phosphatase levels combined with positive antimitochondrial antibodies (AMA) found in 95% of cases provides diagnostic confirmation, even before symptoms develop.

Primary biliary cholangitis (PBC) (formerly called Primary Biliary Cirrhosis) is a liver disease that damages the bile ducts in your liver. These ducts play a crucial role in carrying bile, a fluid that helps with digestion, away from your liver. When the bile ducts don't work properly, substances like fats, cholesterol, and worn-out red blood cells stay in your liver, causing inflammation and scarring. PBC is found most often in women.

Symptoms of PBC

In the early stages of PBC, you may not notice any symptoms. As the disease progresses, you might experience:

  • Fatigue

  • Itchy skin

  • Dry eyes and mouth

  • Yellow skin and eyes (jaundice)

  • Swollen feet and ankles

  • Right upper quadrant abdominal discomfort

  • Swelling in your abdomen

  • Bone, muscle, or joint pain

  • Darkened skin

  • Xanthelasmas or xanthomas (cholesterol deposits in your skin or tendons)

  • Pale stools

  • Dark urine

Causes of PBC

The exact cause of PBC is unknown, but it may be linked to immune system problems like thyroid trouble, pernicious anemia, or scleroderma. External factors such as smoking, chemicals, or infections can also trigger the disease. While PBC isn't passed down from parents to children, there seems to be a family link, and it's more common if you have a parent or sibling with the condition.

Understanding Primary Biliary Cholangitis (PBC): Symptoms, Causes, and Treatment Options

Diagnosing PBC

If you have any symptoms of PBC, your doctor may recommend blood tests to assess your liver function and check for immune system issues. They may also suggest imaging tests like an ultrasound, CT scan, MRI, or endoscopy (ERCP) to get a more detailed look at your liver. In some cases, a liver biopsy may be necessary to examine liver tissue for damage or disease.

Treatment Options for PBC

Although there's no cure for PBC, medications can slow down the disease's progression. The most common drug is ursodiol (ursodeoxycholic acid), which helps move bile through your liver. Another medication, obeticholic acid (Ocaliva), can be used with ursodiol or alone to treat PBC. Newer drugs are also available to treat symptoms like itching and fatigue.

In addition to medications, your doctor may suggest lifestyle changes, such as:

  • Stopping alcohol consumption to reduce strain on your liver

  • Choosing low-sodium foods

  • Exercising regularly to ease symptoms

When medications are no longer effective, a liver transplant may be an option for some patients with advanced PBC.

Additional Resources

For more information on primary biliary cholangitis, visit:

The Bottom Line

Early detection and treatment with UDCA can dramatically slow progression and prevent the need for liver transplantation in most patients. The key is recognizing symptoms like unexplained itching or fatigue, especially in middle-aged women with other autoimmune conditions. If you're experiencing persistent symptoms that could indicate liver disease, Doctronic can help you understand your risk and next steps.

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