Primary Biliary Cholangitis (PBC)
A slow autoimmune disease of the small bile ducts inside the liver, mainly in women. Treated with daily ursodeoxycholic acid; new drugs available for non-responders.
Category
Chronic
Red flags
1
This article is educational and does not replace your doctor's advice. Seek urgent care for any red-flag symptoms.
What is it?
PBC (formerly primary biliary cirrhosis) is a slow autoimmune attack on the small bile ducts inside the liver. Bile builds up and damages liver cells. Without treatment, decades of damage can lead to cirrhosis. With treatment started early, most patients live normal lives.
How common is it?
Rare — about 30-40 per 100,000. 90% are women, usually diagnosed 40-60 years old.
Why does it happen?
Genetic predisposition + environmental triggers. The signature blood marker is anti-mitochondrial antibody (AMA).
Symptoms
- Often silent for years
- Fatigue (most common, can be severe)
- Itching (intense, often worst at night)
- Dry eyes and dry mouth (Sicca syndrome — overlaps with Sjögren's)
- Late: jaundice, easy bruising, fluid retention
Red flags — seek urgent medical care
Jaundice
Indicates advanced disease — needs urgent specialist care.
How is it diagnosed?
Blood tests: elevated ALP and GGT, positive anti-mitochondrial antibody (AMA), elevated IgM. FibroScan for fibrosis. Liver biopsy not always needed.
What the latest science says
Obeticholic acid (Ocaliva) and PPAR agonists (elafibranor, seladelpar) are second-line options for ursodeoxycholic acid non-responders. Improving outcomes substantially.
Outlook — what to expect
With early ursodeoxycholic acid treatment, most patients have normal life expectancy. About 30% don't respond well to ursodeoxycholic acid alone and need second-line drugs. Cirrhosis develops in untreated or refractory cases.
Treatment
Habits & Lifestyle
Address quality-of-life symptoms early.
- Take ursodeoxycholic acid every day.
- Avoid alcohol.
- Get hepatitis A and B vaccines.
- Bone density check — at risk of osteoporosis.
- Vitamin A, D, E, K levels — fat-soluble vitamins are often low.
Diet
Mediterranean diet; supplement what you lack.
- Calcium and vitamin D for bone health.
- Fat-soluble vitamins if low.
Medications
Ursodeoxycholic acid is first-line for everyone.
- Ursodeoxycholic acid (UDCA) 13-15 mg/kg daily — first-line, lifelong.
- Obeticholic acid for non-responders.
- PPAR agonists (elafibranor, seladelpar) — newer options.
- Itching: cholestyramine, rifampin, naltrexone.
- Calcium + vitamin D supplements.
Procedures & Surgery
Procedures for advanced disease.
- FibroScan annually.
- Liver transplant for advanced cirrhosis or intractable itching.
Questions to ask your doctor
Save this list or print it before your appointment — doctors appreciate prepared patients.
- Am I responding well to UDCA — based on what blood tests?
- Should I have a bone density scan?
- Do I need second-line therapy?
Medically reviewed by: SGA Hepatology Panel · Last reviewed 24/04/2026