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Primary Biliary Cholangitis (PBC)

Autoimmune Chronic Cholestatic Liver Disease

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

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 (PBC)?

Primary biliary cholangitis (formerly primary biliary cirrhosis) is a chronic autoimmune disease predominantly affecting middle-aged women, characterized by progressive destruction of small intrahepatic bile ducts.

Diagnosis requires two of three criteria: elevated alkaline phosphatase, positive antimitochondrial antibody (AMA) at 1:40 or higher, and compatible liver histology.

Untreated disease progresses to cirrhosis, portal hypertension, and liver failure over 10–20 years.

Associated conditions include Sjögren syndrome, autoimmune thyroid disease, scleroderma, and celiac disease.

Symptoms

Chronic fatigue (often disproportionate to liver disease severity)
Pruritus, often worse at night and palms/soles
Jaundice, dark urine, and xanthomas/xanthelasma
Right upper quadrant discomfort and hepatomegaly
Sicca syndrome (dry eyes and mouth)
Features of cirrhosis in advanced disease: ascites, varices, encephalopathy

Risk Factors

Female sex (female-to-male ratio 9:1)
Age 30–65 years at presentation
Family history of PBC or other autoimmune disease
Coexisting Sjögren syndrome, autoimmune thyroiditis, or rheumatoid arthritis
Urinary tract infections (molecular mimicry)
Smoking and specific environmental exposures

When to See a Doctor?

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

  • Persistently elevated alkaline phosphatase with cholestatic pattern
  • Unexplained pruritus or fatigue, especially in middle-aged women
  • Signs of advanced liver disease: jaundice, ascites, bleeding

Treatment Methods

01
Ursodeoxycholic acid (UDCA) 13–15 mg/kg/day — first-line, improves biochemistry and survival
02
Assess biochemical response at 12 months: POISE criteria, Paris II, or Toronto criteria
03
Obeticholic acid 5–10 mg/day or fibrates (bezafibrate, fenofibrate) as second-line add-on for inadequate UDCA response
04
Symptom management: cholestyramine, rifampicin, sertraline, or naltrexone for pruritus
05
Surveillance: fat-soluble vitamin (A, D, E, K) replacement, bone density scans, HCC screening in cirrhosis
06
Refer for liver transplantation at MELD over 15, refractory symptoms, or decompensation

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.

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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.