Primary biliary cholangitis

Overview

Primary biliary cholangitis (PBC) is a long-term liver disease that slowly damages the bile ducts, which are small tubes that carry bile out of the liver. As bile builds up in the liver, it can cause scarring (cirrhosis) and eventually liver damage.

No, PBC is not very common. It affects about 1 in 5,000 people in the UK.

PBC most often affects women between the ages of 30 and 60, but it can also occur in men and at other ages.

Symptoms

Common symptoms

• Extreme tiredness (fatigue)
• Itchy skin (itching often worse at night)
• Dry eyes and dry mouth (called sicca syndrome)
• Yellowing of the skin and eyes (jaundice) – usually a later sign
• Pain in the upper right side of the belly
• Dark urine and pale stools

Symptoms in children

• PBC is very rare in children, but if it occurs, common symptoms include persistent itching, fatigue, and failure to grow or gain weight as expected.

Symptoms in older adults

• In older adults, symptoms are similar to those in younger adults, but fatigue and cognitive issues (like memory problems) may be more noticeable.

Causes

Main causes

• The exact cause is not fully understood. PBC is thought to be an autoimmune disease where the immune system mistakenly attacks the bile ducts.
• Genetics may play a role, as PBC can run in families.

Risk factors

• Being female
• Being between the ages of 30 and 60
• Having a family history of PBC or other autoimmune diseases (like lupus or thyroid disease)
• Smoking
• Certain infections (possibly involved, but not proven)

When to see a doctor

See a doctor urgently if:

• If you have persistent jaundice (yellow skin or eyes)
• Unexplained weight loss
• Severe itching that does not improve
• Swelling of your legs or abdomen (possible fluid buildup)

Book a routine appointment if:

• If you have ongoing tiredness, dry eyes or mouth, or other symptoms that concern you
• If you have a family history of PBC and want to discuss screening
• At any routine check-up if you have known risk factors

Diagnosis

Your doctor will ask about your symptoms and medical history, do a physical exam, and arrange tests. The process is usually done as an outpatient.

Tests that may be done

• Blood tests – to check liver function and look for specific antibodies (anti-mitochondrial antibodies, or AMA)
• Ultrasound scan of the liver to see its structure
• Liver biopsy (removing a tiny sample of liver tissue) – sometimes needed to confirm the diagnosis and check for scarring

What to expect at your appointment

You will likely be referred to a liver specialist (hepatologist). The tests are usually painless or cause only mild discomfort. A biopsy is done with a local anaesthetic, and you may need to rest afterwards.

Treatment

There is no cure for PBC, but treatments can slow the disease, manage symptoms, and help you live well. Early treatment is important.

Self-care at home

• Avoid alcohol completely – it puts extra strain on your liver.
• Do not smoke.
• Get vaccinated against hepatitis A and B (these infections can be more serious if you have liver disease).
• Maintain a healthy weight with a balanced diet and regular, gentle exercise.
• For itching: use moisturisers, cool baths, and avoid hot showers. Ask your doctor about anti-itch creams.

Medical treatments

Your doctor may prescribe medications that help reduce bile acid buildup and slow liver damage. Other medicines can help with itching and fatigue. You may also receive vitamin supplements if you are deficient. Always take treatments as directed and discuss any side effects with your healthcare team.

When is surgery considered?

In advanced PBC where the liver is severely damaged, a liver transplant may be considered. This is a major operation but can be life-saving. Your doctor will discuss if this is an option for you.

Living with this condition

Living with PBC often means managing fatigue and itching. It helps to pace yourself, plan rest breaks, and listen to your body. Regular check-ups with your liver specialist are key to monitoring your condition.

Lifestyle tips

• Avoid alcohol and smoking.
• Manage stress with relaxation techniques, hobbies, or talking to friends.
• Stay connected with people who support you.
• Get enough sleep and rest when you need it.

Diet and exercise

Eat a balanced diet rich in fruits, vegetables, and whole grains. Limit salt if you have fluid retention. Stay active with gentle activities like walking, swimming, or yoga, but don't overdo it. Ask your doctor for personalised advice.

Mental health and emotional wellbeing

Living with a chronic illness can lead to anxiety, sadness, or stress. It is important to talk about how you feel – with family, friends, a counsellor, or a support group. If you ever have thoughts of harming yourself, call your local emergency number or a crisis line immediately.

Prevention

There is no known way to prevent PBC because the cause is not fully understood.

Vaccines

Yes – vaccinations for hepatitis A and hepatitis B are recommended for people with PBC to protect the liver from further damage.

Screening programmes

Routine screening is not generally recommended, but if you have a family history of PBC or other autoimmune diseases, discuss your risk with your doctor.

Complications

If left untreated

• Scarring of the liver (cirrhosis)
• Liver failure (the liver stops working properly)
• High blood pressure in the liver's veins (portal hypertension)
• Bone thinning (osteoporosis)
• Vitamin deficiencies (especially A, D, E, and K)
• Increased risk of liver cancer

Long-term outlook

With early diagnosis and proper treatment, most people with PBC can manage their symptoms and live a full life for many years. The disease usually progresses slowly, and medications can help slow it down. In advanced stages, a liver transplant is very successful. Regular medical care gives you the best chance at a good outcome.

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