Primary biliary cholangitis, usually shortened to PBC, is a long-term autoimmune condition in which the immune system slowly damages the smallest bile ducts inside the liver, so that bile backs up and, over many years, can scar the liver [1].
This page provides general information. It does not replace advice from a doctor or another qualified healthcare professional.
The liver makes a greenish-yellow fluid called bile, which helps digest fat and carries waste away. Bile leaves through a branching network of small tubes called bile ducts [8]. In PBC the immune system treats the cells lining the smallest of those ducts as foreign and destroys them [6]. Drainage worsens, bile builds up, and that buildup, called cholestasis, irritates nearby liver cells, causing inflammation and, over years, scarring. Heavy scarring is called cirrhosis, and most people with PBC never reach that point [1,6].
The name. Until 2015 this condition was called primary biliary cirrhosis. Patient organizations and liver specialists campaigned to change it, because the word cirrhosis wrongly implied that everyone already had advanced scarring, which most do not. It also carried an unfair link with heavy drinking and caused real problems with insurance [6,7]. Cholangitis means inflammation of the bile ducts, which is accurate [6]. Many people still search the old name, and both refer to the same condition [1].
PBC differs from primary sclerosing cholangitis, which affects larger ducts, and from autoimmune hepatitis, which attacks liver cells [2,3].
PBC is rare, and affects far more women than men, in ratios reported from about nine to one down to nearer four to one [2,6]. A 2026 review pooling 59 studies across 25 countries put the worldwide figure at about 18 people per 100,000, roughly 1 in 5,500, with about 1.8 new cases per 100,000 people each year [4]. Prevalence counts people living with it; incidence counts new diagnoses each year. Both count diagnosed people only, so anyone not yet identified is missing. Most people are diagnosed in middle age [1].
Reported numbers have risen over recent decades, most steeply in the Western Pacific region [4,10]. Part of that rise is genuine. A large part is better detection, since routine blood tests are far more common and the confirming antibody test has grown more sensitive [3,10].
Rates are higher further from the equator and in wealthier countries [4]. About 35 people per 100,000 are affected in north-east England, and around 22,000 across the United Kingdom [3,6]. United States data for 2014 found about 58 per 100,000 women and 15 per 100,000 men [1]. Reliable figures for much of Africa, South America and the Middle East are not available.
Nobody knows why PBC starts. The accepted explanation is that a person inherits genes that make their immune system more likely to react this way, and something in the environment then triggers it [1,10]. Genes are the instructions inside our cells. There is no genetic test for PBC [6]. Triggers studied include infections, smoking, certain chemicals and changes in gut bacteria [1,10].
PBC is not inherited in a simple pattern, is not contagious and is not caused by alcohol. A close relative with PBC slightly raises the chance, most noticeably between mothers and daughters, though the risk stays small, and testing well relatives is not routinely recommended [6].
Many people with PBC also have another autoimmune condition, most often a thyroid problem, Sjögren’s syndrome or scleroderma [1].
Around 6 in 10 people are diagnosed before any symptoms appear, usually after a routine blood test showed an unexpected liver result [1]. Two symptoms shape daily life most [1,2]:
Others include dry eyes and mouth, aching joints and small yellow fatty bumps under the skin [1,8].
Neither fatigue nor itching shows how damaged the liver is. Someone can feel exhausted and itch constantly with an early-stage liver, while someone with more scarring feels well. Guidelines say plainly that relieving symptoms is as important as protecting the liver [2,3,5].
When to seek urgent help. Most people with PBC will never face an emergency. Contact a doctor without delay if there is vomiting of blood or black tarry stools, sudden confusion, new or worsening yellowing of the eyes or skin, or rapid swelling of the abdomen [1].
Diagnosis usually starts with a blood test ordered for another reason. The result that raises suspicion is a raised alkaline phosphatase, an enzyme that climbs when bile drainage is blocked [1,3]. The second test looks for antimitochondrial antibody, written AMA, found in about 95 out of 100 people with PBC and rarely in anyone else [1,2].
A diagnosis is generally made when at least two of three features are present: blood tests showing blocked bile drainage, AMA in the blood, and, if a biopsy has been done, the typical pattern of bile duct damage [2]. The blood tests alone confirm it in around 9 out of 10 people, so most people never need a liver biopsy, and guidelines advise against one routinely because it carries a small risk of bleeding [5]. Biopsy is kept for those whose antibody tests are negative, or where autoimmune hepatitis may also be present [2,5].
Care is normally shared with a liver specialist, called a hepatologist [3]. Some people carry AMA with normal liver tests, which is not PBC [5].
There is no cure. Treatment has two equally important aims: slowing liver damage, and reducing symptoms [5].
First-line treatment. Ursodeoxycholic acid, often called urso or UDCA, is recommended for everyone with PBC at 13 to 15 mg for each kilogram of body weight per day [2,5]. It improves bile flow and is taken lifelong. People whose blood tests respond well have life expectancy close to that of the general population [5,6]. Dose is set by weight, so weight is rechecked and the dose adjusted [5].
The twelve-month check. Blood tests are repeated after about a year, and roughly 20 to 40 out of every 100 people do not respond well enough [2,6]. An alkaline phosphatase still above about 1.67 times the upper limit of normal, or a raised bilirubin, marks higher risk of progression and usually triggers adding a second medicine [2,3]. Risk scores such as the UK-PBC score and the Global PBC score estimate risk over time [3,5]. Specialists increasingly aim for a fully normal alkaline phosphatase [10].
Second-line medicines. Elafibranor and seladelpar, two once-daily tablets called PPAR agonists, are now approved as add-ons. Elafibranor gained accelerated United States approval in June 2024 and conditional European authorization that September; seladelpar followed in August 2024 and February 2025 [15,16,17,18,19,23]. NICE recommends both for the NHS in England [20,21]. Both were cleared on improved blood tests, so longer survival has not been shown and confirmatory studies are running [16,18]. Bezafibrate, a cholesterol medicine, is used off-label in some countries, with trial evidence that it improves blood tests and itching [22].
Obeticholic acid: an important change. The first second-line medicine for PBC still appears in older guidance and leaflets, and is no longer available in the United States or the European Union. The European Commission revoked its authorization in August 2024, and the United States Food and Drug Administration withdrew approval from 24 November 2025, after the required follow-up study failed to confirm a benefit [11,13]. Professional bodies are updating their guidance [12]. It stayed licensed elsewhere while regulators reviewed it, so anyone taking it should ask their liver team [14].
Symptoms. For itch, cholestyramine, a powder that binds bile acids in the gut, is usually tried first, taken well apart from other medicines, which it otherwise blocks [2,5]. Low-dose rifampicin is the next step, with blood tests to watch the liver [2,5]. Linerixibat became the first medicine approved specifically for PBC itch in the United States in March 2026 [25]. Persistent itch should lead to specialist referral [5]. Fatigue has no single treatment; the advice is to look for other causes such as a thyroid problem, anemia or poor sleep, then work on pacing and support [2,5].
Liver transplant is considered when the liver is failing, and results in PBC are better than for most other liver conditions [2].
Monitoring continues for life, usually blood tests once or twice a year plus checks for related problems [3,6]. Bone thinning is common, so bone density is monitored and calcium, vitamin D or bone-protecting medicines may be advised [1,3]. Cholesterol is often raised and statins can be used safely [1,3]. In more advanced disease, vitamins A, D, E and K may be needed [1,3]. Dry eyes and mouth deserve their own treatment [1,5].
Urso neither reduces the chance of becoming pregnant nor harms a pregnancy, though pregnancy is worth planning with the liver team in advance [5]. Not smoking, limiting alcohol and staying active all help [1,8]. Guidelines recommend that everyone with PBC be offered contact with a patient support organization, a first for a liver guideline [5].
Clinical trials test whether a treatment works and is safe. PBC is an active research area, so studies do come up. Deciding whether to look into one is personal, and it helps to take it in steps.
It is worth being clear on what a study involves:
Every trial has rules about who can take part, called eligibility criteria. Some describe who can join, others who cannot. For a PBC study they might include a confirmed diagnosis, a year on a stable dose of urso, an alkaline phosphatase within a set range, no decompensated cirrhosis, and an itch score above a threshold.
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Study status checked: 3 August 2026. Research moves quickly and what is studied changes, so this list will date. For current information, search at app.trialport.com.
The PBC Foundation in Edinburgh is the main international organization for this condition, with a helpline, material in nine languages and members in more than 90 countries [6].
In the United Kingdom, Liver UK, formerly the British Liver Trust, has a reviewed PBC section, a booklet and a free nurse-led helpline [7].
In the United States, the PBCers Organization is a patient-led group whose president speaks publicly for the community [25]. In Canada, Liver Canada publishes information in English and French [8]. The NIDDK covers PBC in English and Spanish [1].
Elsewhere coverage is thinner, with a general liver charity rather than a PBC group, so the PBC Foundation is often the best route in.
Is primary biliary cholangitis the same as cirrhosis?
No. Cirrhosis means advanced scarring, and most people with PBC never develop it. That confusion is why the name changed in 2015 [6,7].
Why am I so tired and itchy when my liver tests look good?
Symptoms do not track liver damage in PBC, which is why guidelines treat symptom relief as a separate goal [2,3,5].
Did drinking cause this, and will my children get it?
No, and almost certainly not. PBC is autoimmune, and the old name was misleading on the alcohol point. Risk is slightly higher in close relatives, particularly daughters of affected mothers, though it stays small [6].
How long will I live with PBC?
People who respond well to urso have life expectancy close to that of the general population, and most people diagnosed today die with PBC rather than from it [5,6].
What happened to obeticholic acid?
Its European authorization was revoked in 2024 and its United States approval withdrawn in 2025, after the follow-up study did not confirm benefit [11,12,13].
Written by: trialport editorial team
Reviewed by: Keith Berelowitz, Founder and CEO, trialport
Reviewed on: 3 August 2026
Next review due: 3 August 2027
References last checked: 3 August 2026