Primary biliary cholangitis (PBC)
Primary biliary cholangitis is a long-term liver disease in which a person's own immune system slowly destroys the smallest bile ducts inside the liver.
On this page
Medicines commonly prescribed for Primary biliary cholangitis (PBC)
For informational purposes only. Always consult a doctor before using any medicine.
Dosage form: TABLET, 450 mgActive substance: ursodeoxycholic acidManufacturer: Glenmark Arzneimittel GmbhPrescription requiredDosage form: TABLET, 150 mgActive substance: ursodeoxycholic acidManufacturer: Glenmark Arzneimittel GmbhPrescription requiredDosage form: CAPSULE, 500 mgActive substance: ursodeoxycholic acidManufacturer: Glenmark Arzneimittel GmbhPrescription required
Primary biliary cholangitis is a long-term liver disease in which a person's own immune system slowly destroys the smallest bile ducts inside the liver. Bile stagnates, damages the tissue around it, and over the years scarring appears where working cells used to be. It affects mostly women, and the diagnosis is usually made between the ages of forty and sixty. The word cholangitis is misleading here: this is not an infection or a purulent inflammation to be treated with antibiotics but an autoimmune process, and it cannot be caught from anyone. Alcohol has nothing to do with causing it either.
What happens to the bile ducts
The liver makes bile, a fluid without which fats and fat-soluble vitamins are not absorbed and through which the body clears some of its waste. From the liver cells bile is gathered into very fine ducts, which merge into larger ones and eventually empty into the bowel.
In this disease immune cells attack the lining of the smallest ducts and gradually destroy them. Bile can no longer find its way out, builds up in the liver and acts as a corrosive substance: it damages neighbouring cells, keeps inflammation going, and connective tissue is laid down where liver tissue has died. That is fibrosis, and its extreme degree is cirrhosis.
Exactly why the immune system behaves this way is not known: a hereditary predisposition combines with some external trigger, such as past infections or smoking. None of them is the cause on its own, so there is no reason to blame yourself for developing the disease.
The old name is worth a mention. For many years the condition was called primary biliary cirrhosis, and that wording still turns up in letters and reports. The change was deliberate: in most people diagnosed today and treated in time cirrhosis never develops, and the word in the diagnosis frightened people and got in the way of work and insurance.
How it makes itself known
Often it does not. A good many people are diagnosed by chance, after blood tests taken for an entirely different reason came back with abnormal liver results. That is good news: the earlier it is found, the better the outlook.
Where there are symptoms, the commonest are:
- exhausting tiredness that does not lift with rest, and daytime sleepiness;
- itching, sometimes all over, sometimes on the palms and soles; it is worse at night, in warmth, under clothing and during pregnancy;
- dry eyes and dry mouth, a gritty feeling under the eyelids;
- an ache or heaviness under the right ribs;
- pain in the bones and joints;
- firm yellowish plaques on the skin, usually around the eyes.
How bad the symptoms are and how damaged the liver is have surprisingly little to do with each other. One person may be worn down by relentless itching with a liver that is still largely intact; another may feel nothing for years with well-established fibrosis. Feeling well is therefore not a reason to give up on monitoring or treatment.
How the diagnosis is reached
The mainstay is a blood test, and in most cases it is enough; two things are looked at together. The first is a biochemistry pattern showing that bile is not flowing: a raised alkaline phosphatase, often alongside gamma-glutamyl transferase. The second is antimitochondrial antibodies, found in almost everyone with the condition and highly characteristic of it. Immunoglobulin M is often raised as well. A persistently high alkaline phosphatase together with those antibodies confirms the diagnosis for practical purposes.
An ultrasound scan is always done, but with a different aim: to make sure the bile is not being held up by a stone, a narrowing or a tumour in the larger ducts, because that is a different situation and is treated differently.
A liver biopsy is not needed by everyone. It is taken when the antibodies are absent but the picture still fits, or when an overlap with autoimmune hepatitis is suspected. The degree of scarring is now more often assessed with elastography, a painless test resembling an ordinary scan that measures the stiffness of the liver.
What is in your hands
There are only a few points, but they genuinely affect the course of the disease.
- Alcohol. It does not cause this condition, but it adds a second burden to the liver. With advanced fibrosis it is given up altogether; otherwise the limit is agreed with a doctor.
- Smoking. It is linked with faster progression, so stopping is not a general piece of advice here but part of the treatment.
- Medicines. A damaged liver handles some drugs less well. Non-steroidal anti-inflammatory drugs are particularly unwelcome in advanced disease: they raise the risk of bleeding and put a strain on the kidneys. Paracetamol is usually allowed, but at a dose agreed with a doctor. Always tell doctors and pharmacists about the diagnosis, including those prescribing for something unrelated, and do not start herbal remedies or supplements on your own — some of them are toxic to the liver.
- Vaccination. It is worth being vaccinated against hepatitis A and hepatitis B: a viral infection costs an already ailing liver more. Schedules and availability follow your country's immunisation programme.
- Diet and weight. Nothing exotic: an ordinary balanced diet, enough protein, an eye on weight. Adding fatty liver disease on top of this one is the last thing needed.
How it is treated
The mainstay is ursodeoxycholic acid, a naturally occurring bile acid given as tablets or capsules. It makes bile less aggressive and helps it drain, slows scarring and improves the blood results. The dose is worked out by body weight, it is taken continuously for years, and it should not be stopped on your own initiative: the results drift back to where they were. It does very little for tiredness and not much for itching, which is worth knowing in advance so as not to be disappointed by a drug that is nonetheless needed.
After about a year the response is assessed on blood tests, chiefly the alkaline phosphatase and the bilirubin. This is the important fork in the road. In most people the response is good, and with it life expectancy is close to normal — probably the single most important point on this page. In some the response falls short, and a second-line drug is then added.
Second-line drugs work along other routes: some on the receptors governing bile acid handling, others on the way the liver deals with fats. Which of them are licensed differs from country to country and keeps changing, so they are chosen by the specialist — a hepatologist or gastroenterologist who looks after such patients. Some are not used in advanced disease, another reason not to make the decision alone.
A liver transplant is considered when the liver stops coping and, less often, for intolerable itching that nothing has relieved. The results are good: most people live many years with a transplanted liver. The disease sometimes returns in the new organ, though it usually behaves more mildly then.
Itching, tiredness and dryness: what to do about them
The itch in this disease does not start in the skin, which is why creams do not settle it: what irritates the nerve endings are substances retained in the blood. The first drug prescribed binds bile acids in the bowel; it comes as a powder to be mixed with water or juice. One practical detail matters: it binds other medicines too, so ursodeoxycholic acid and everything else are taken separately from it, several hours apart. If that is not enough, other options are added — an antituberculosis antibiotic that works here against itch, an opioid receptor blocker, or certain antidepressants. Simple things help as well: cool showers, loose cotton clothing, moisturisers, short nails and a cool bedroom.
Tiredness is the commonest symptom and the most stubborn; there is as yet no medicine aimed at it. Before putting it down to the disease it is worth checking the things that can be treated: anaemia, an underactive thyroid, disordered breathing in sleep, depression. After that what works is pacing: heavy tasks in the first half of the day, planned pauses, regular activity within your limits, and steady sleep times.
For dry eyes and mouth, artificial tears and saliva substitutes help. A dry mouth noticeably raises the risk of tooth decay, so dental visits should be more frequent than usual; between meals, sips of water and sugar-free gum are useful.
What is watched besides the liver
The disease rarely comes alone, and some of its consequences develop well away from the liver.
- Bones. When bile is not flowing, vitamin D is absorbed less well, bones thin and fractures follow minor falls. Bone density is measured with a DEXA scan, and vitamin D with calcium is prescribed if needed, sometimes along with drugs for osteoporosis.
- Fat-soluble vitamins. With marked cholestasis, vitamins A, D, E and K are absorbed less well. A lack of vitamin K shows itself as easy bruising and bleeding. They are replaced according to test results, not on spec.
- Cholesterol. It is often high and that is alarming, but in this condition the rise does not carry the same increase in cardiovascular risk as it usually would. The decision to treat is made on the whole picture rather than on a single figure.
- Neighbouring autoimmune conditions. The commonest are an underactive thyroid, Sjögren's syndrome, coeliac disease, scleroderma and Raynaud's phenomenon. They are looked for when the matching symptoms appear.
- Monitoring in cirrhosis. If scarring has reached cirrhosis, regular ultrasound scans are added to pick up liver cancer early, along with an endoscopy to look for varices in the oesophagus.
When it cannot wait
The disease itself moves slowly, but it has sudden turns. Call an ambulance (in Spain, Italy, Portugal, Poland and Ukraine, 112) if there is:
- vomiting of blood, or of dark material like coffee grounds;
- black, tarry stools;
- confusion, marked drowsiness, disorientation or odd behaviour;
- a coarse flapping tremor of the hands with the arms held out, together with drowsiness;
- severe abdominal pain with a high temperature and shivering.
Seek advice the same day or within a few days if:
- the whites of the eyes or the skin turn yellow, and the urine darkens while the stools go pale;
- the abdomen starts swelling quickly and the legs become puffy;
- a fever appears with no clear cause;
- the itching becomes unbearable and keeps you awake;
- bruises appear or the gums bleed without any injury.
Online consultation
This condition is managed mainly through blood tests and conversation, which makes it a good fit for an online appointment. The doctor will go through your biochemistry over time, explain what an alkaline phosphatase above the normal range means and why it is tracked, and judge whether the response to treatment is good enough or whether it is time to discuss the next step with a specialist.
Everyday questions are easily settled online, the sort not worth losing a day of travelling for: how to space out your medicines, what to do about itching that has worsened, whether a particular painkiller is allowed, which vaccinations you need. Have your recent blood tests ready along with older ones for comparison, the ultrasound or elastography reports, a full list of drugs and supplements with doses, and brief notes on how you are: when the itch is worst, how you sleep, how far your energy goes through the day.
An online appointment does not do away with being seen in person. Examination, ultrasound, bone density scanning and endoscopy happen only face to face, and if the doctor says it is time, that is part of the monitoring plan rather than a formality.
This material is for information only and does not replace medical advice.
Consult with a doctor about Primary biliary cholangitis (PBC)
Discuss your symptoms and possible next steps with a doctor online.
Online doctors for Primary biliary cholangitis (PBC)
Discuss your symptoms and possible next steps for Primary biliary cholangitis (PBC) with a doctor online.
