Primary Biliary Cholangitis

Autoimmune liver disease with progressive destruction of intrahepatic bile ducts.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 10, 2026

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Primary Biliary Cholangitis?

Primary biliary cholangitis (PBC, formerly primary biliary cirrhosis) is a chronic cholestatic liver disease that develops with autoimmune destruction of small intrahepatic bile ducts. It is most common in middle-aged women, with a female-to-male ratio of approximately 9:1.

Anti-mitochondrial antibody (AMA) is positive in 95% of patients and has high diagnostic value. Elevated alkaline phosphatase and GGT are typical laboratory findings. Histology shows granulomatous inflammation around the bile ducts (florid duct lesion).

Ursodeoxycholic acid (UDCA) forms the cornerstone of treatment and significantly slows disease progression when started early. Obeticholic acid or bezafibrate may be added in cases of inadequate response to UDCA.

Symptoms

Fatigue (the most common and prominent symptom)

Pruritus (due to cholestasis — particularly at night)

Jaundice (in advanced stage)

Dry mouth and dry eyes (Sjögren's syndrome association)

Xanthelasma and xanthomas (cholesterol deposits)

Right upper quadrant pain

Risk Factors

Female sex

Family history

Other autoimmune diseases (Sjögren's, thyroiditis, celiac)

Smoking

Recurrent urinary tract infections (possible trigger)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

If persistent itching and fatigue are present
If a cholestatic pattern is detected in liver enzymes
If AMA positivity is found
If jaundice or signs of cirrhosis develop

Treatment Methods

Ursodeoxycholic acid (UDCA) — 13-15 mg/kg/day (lifelong)

Obeticholic acid (in inadequate response to UDCA)

Bezafibrate (combined with UDCA — may provide added benefit)

Pruritus treatment: Cholestyramine, rifampin, naltrexone

Bone loss prophylaxis (vitamin D, calcium, bisphosphonates)

Liver transplantation in advanced stage

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Dahiliye (İç Hastalıkları) Department

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Primary Biliary Cholangitis (PBC)

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Primary biliary cholangitis is a chronic autoimmune cholestatic liver disease causing progressive destruction of small intrahepatic bile ducts with elevated alkaline phosphatase and positive antimitochondrial antibody; ursodeoxycholic acid is first-line, with obeticholic acid or fibrates as second-line for inadequate response.

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Hepatitis C (HCV)

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.