Primary biliary cholangitis (PBC): symptoms, treatment and outlook

The NHS explains that bile is a liquid produced in the liver that helps digest fats, absorb certain vitamins and remove waste products. It passes out of the liver through small tubes called bile ducts. In PBC the immune system mistakenly attacks the bile ducts. They become damaged and injured, which causes bile to build up in the liver, further damaging it and potentially leading to scarring (cirrhosis).

The PBC Foundation notes that PBC was formerly known as primary biliary cirrhosis, and was renamed in 2015 because most people with PBC never develop cirrhosis. The NHS still lists the older name in brackets. It is not caused by alcohol, and it is not infectious. The PBC Foundation says it cannot be passed on by normal contact, by blood, sex or breastfeeding.

The NHS says PBC does not always cause symptoms, and that some people have symptoms early on while others develop them later. Symptoms can range from mild to severe, and this is not always related to the degree of liver damage.

• Fatigue. A common symptom, though not always caused by PBC, that can have a significant impact on daily activities.

• Itchy skin, which can be widespread or affect one area, and may be worse at night, in contact with fabrics, when warm, or during pregnancy.

• Dry eyes and mouth.

• Bone or joint aches.

• Feeling very sleepy during the day.

• Pain or discomfort in the upper right side of the tummy.

The NHS says that as the liver becomes more scarred and damaged, eventually leading to cirrhosis, additional symptoms can occur. These include yellowing of the whites of the eyes and skin (jaundice), fluid build-up in the legs, ankles and feet, fluid build-up in the tummy (ascites), small fatty deposits on the skin usually around the eyes (xanthelasmata), dark urine and pale stools, easier bleeding and bruising, and problems with memory and concentration.

The NHS lists potential complications if PBC is not treated or reaches an advanced stage: osteoporosis, portal hypertension, ascites, vitamin deficiencies (A, D, E and K) and a slightly increased risk of liver cancer.

The NHS says many people are only diagnosed with PBC after having a routine blood test for another reason, and that PBC can usually be diagnosed just using blood tests. Once PBC is diagnosed, you will also need an ultrasound scan to help rule out other problems with the bile ducts and assess the liver. A liver biopsy is occasionally recommended to assess the liver and help doctors decide on the best treatment.

The NHS says the aim of treatment is to slow down the liver damage and reduce your symptoms. Ursodeoxycholic acid (UDCA) is the main treatment. It can help delay liver damage in most people, particularly if you start taking it in the early stages, but it does not improve symptoms such as fatigue. Once you start UDCA, it is likely you will need to take it for the rest of your life. You will be assessed after a year to see whether you have responded.

For itch, the NHS says colestyramine is widely used. If itching does not improve, see a GP, as alternatives such as rifampicin and naltrexone are usually prescribed by the specialist. Regularly using moisturisers to stop the skin becoming dry may also help.

No medicines are currently available specifically to treat fatigue in PBC. A GP may want to rule out other causes such as iron deficiency anaemia, sleep apnoea or depression. The NHS suggests trying to exercise whenever possible while pacing yourself, adjusting your routine around fatigue, which is often worse later in the day, and using sleep hygiene measures.

For dry mouth and eyes, a GP may recommend artificial tears or saliva substitutes. The NHS advises taking care of your teeth and gums and visiting a dentist regularly because a dry mouth can increase the risk of tooth decay.

The NHS says all types of liver disease, including PBC, can be helped by following general health advice: stop smoking, eat a balanced diet, and stop drinking alcohol or drink only a small amount depending on how much liver damage you have. The damage to your liver can affect how it processes some medicines, so you may need to avoid certain ones such as aspirin and ibuprofen. Tell any pharmacist or healthcare professional treating you that you have PBC.

The NHS says PBC is a progressive condition, which means the damage to the liver can steadily get worse over time, and the rate varies between individuals and can sometimes take decades. Without treatment, the liver can become so badly damaged that it no longer works properly, which is known as liver failure and can be fatal. Current treatments can help slow down the liver damage.

The PBC Foundation says that people who respond to UDCA are thought to rejoin the general population in terms of life expectancy, and that approximately 20 to 30% of people do not respond effectively and need closer monitoring. It says most people will die with their PBC rather than because of it.

See a GP if you have symptoms such as persistent itch, fatigue or yellowing of the skin or eyes. If you have PBC with cirrhosis, the NHS says to ask for an urgent GP appointment or get help from NHS 111 if you have yellow skin or eyes, difficulty breathing or a swollen tummy, legs, ankles or feet, and to call 999 or go to A&E if you are vomiting blood, your poo is very dark or black, or you suddenly become confused or have slurred speech.

On the NHS, PBC is usually looked after by a hepatologist or gastroenterologist, with your GP helping with symptoms. The NHS says people who may need obeticholic acid, elafibranor or seladelpar first need to be assessed by a specialist team with experience in treating PBC. Some people also see a private liver specialist for consultation and tests.