Primary biliary cholangitis

Also called PBC, Primary biliary cirrhosis

An autoimmune liver condition, mostly in women. Itching and fatigue are the symptoms, and the itch is treatable when anybody asks about it.

What it is

An autoimmune condition in which the small bile ducts within the liver are gradually destroyed, causing bile to build up and damage the liver.

It affects women around nine times more often than men, usually beginning between 40 and 60. It was renamed from primary biliary cirrhosis because most people never develop cirrhosis, and the old name caused unnecessary alarm.

It is often found by accident, from a raised alkaline phosphatase on a routine blood test, and confirmed by an anti-mitochondrial antibody test.

**Treatment changes the course.** Ursodeoxycholic acid, taken daily, slows progression and, in people who respond, gives close to normal life expectancy. Second-line drugs exist for those who do not respond. Starting treatment early matters, which is an argument for not ignoring an unexplained raised alkaline phosphatase.

The two symptoms that dominate life are fatigue and itching, and neither correlates with how bad the liver disease is. **The itch is treatable**, with specific drugs rather than antihistamines, and it is chronically under-treated because clinicians focus on liver numbers and do not ask.

Bone thinning is common, and everybody should have bone density assessed.

Around a third of people also have Sjögren's syndrome, and thyroid disease is common alongside.

Signs you might notice

Often nothing, with the diagnosis made from a blood test.

Fatigue, frequently profound, and not proportional to the liver findings.

Itching, often worse at night, sometimes intense and unbearable.

Dry eyes and dry mouth.

Discomfort under the right ribs.

Later: jaundice, fatty deposits around the eyes, dark urine and pale stools.

Advanced disease: swelling, easy bruising, confusion, or vomiting blood. See cirrhosis.

Urgent: vomiting blood, black stools, new confusion, or a swollen abdomen with fever.

Ask for investigation of any persistently raised alkaline phosphatase without an obvious cause.

How it can affect day-to-day life

Fatigue is the symptom people rate as most disabling and the one least addressed. It is not proportional to liver function and is not simply depression, and pacing and occupational therapy input helps.

The itch is a specific treatable symptom. Antihistamines mostly do not work; the drugs that do are colestyramine and, beyond that, rifampicin and others under specialist care. Nobody should be enduring an unbearable itch without those having been tried.

Fat-soluble vitamin deficiencies develop, so vitamins A, D, E and K should be checked.

Bone density scanning and treatment for osteoporosis is standard and often missed.

Six-monthly liver cancer surveillance is recommended where cirrhosis has developed.

Because it is more common in women in midlife, symptoms are frequently attributed to the menopause, which delays diagnosis.

Supporting someone well

Do not ignore an unexplained raised alkaline phosphatase; ask for an anti-mitochondrial antibody test.

Take ursodeoxycholic acid consistently; it is what alters the course.

Ask about the response to treatment at 12 months and about second-line drugs if the response is poor.

Ask for the itch to be treated properly, starting with colestyramine, taken away from other medicines.

Ask for bone density scanning and treatment.

Ask for fat-soluble vitamin levels to be checked.

Ask about liver cancer surveillance if cirrhosis is present.

Get tested for thyroid disease and for Sjögren's syndrome.

Take fatigue seriously and ask for pacing support rather than accepting it.

Where to get help

A GP practice for blood tests and referral to hepatology.

A specialist liver service for treatment and monitoring.

British Liver Trust on 0800 652 7330, who have a PBC support network.

Sjogren's UK for the associated dry eyes and mouth.