Primary biliary cirrhosis - Complications of primary biliary cirrhosis

If primary biliary cirrhosis (PBC) is not treated or reaches an advanced stage, it can cause other potentially serious problems.

Osteoporosis

For reasons that are unknown, people with PBC are at an increased risk of developing osteoporosis. This is a condition in which the bones become weak and brittle.

A DEXA scan, which is a type of X-ray that measures the density of your bones, may be carried out when you are diagnosed with PBC and regularly thereafter to monitor the condition of your bones.

Your GP may prescribe vitamin D and calcium supplements to help maintain your bone strength.

You may also be advised to exercise regularly, with a particular focus on weight-bearing exercises such as running, dancing and aerobics, because this can help reduce your risk of osteoporosis.

In some cases, you may also need more specific treatment, such as bisphosphonates. This is a medication that slows the rate at which bone is broken down by the body, which can help maintain bone density and reduce the risk of a fracture.

Read more about treating osteoporosis and preventing osteoporosis.

Portal hypertension and varices

Portal hypertension is increased blood pressure inside your liver as a result of damage and scarring (cirrhosis), making it harder for blood to move through it.

For your blood to return from your liver to your heart, new blood vessels start to open up, usually along the lining of your stomach or oesophagus (the long tube that carries food from the throat to the stomach). These new blood vessels are known as varices.

The walls of these varices are quite fragile and in some cases can split open and bleed.

This can cause gradual, long-term bleeding, which can lead to anaemia (a lack of red blood cells), or it can be sudden and severe, causing you to vomit blood and pass stools that are very dark or tar-like.

If you vomit blood or have very dark stools, you should go to your local GP surgery or your nearest accident and emergency (A&E) department immediately.

Portal hypertension can sometimes be managed with a type of medication known as beta blockers, which can help reduce your blood pressure, although a procedure to seal the varices using an endoscope (a narrow tube with a camera at the end that is passed down your throat) may be recommended in some cases.

Ascites

If you have portal hypertension, you may also develop a build-up of fluid in your abdomen (stomach) and around your intestines. This fluid is known an ascites.

Initially, this can be treated with water tablets (diuretics). If the problem progresses, many litres of fluid can build-up and this will need to be drained. This is a procedure known as paracentesis and involves a long thin tube being placed into the fluid through the skin under local anaesthetic.

One of the problems associated with ascites is the risk of infection in the fluid (spontaneous bacterial peritonitis). This is potentially a very serious complication and can be fatal.

Hepatic encephalopathy

One of the most important functions of the liver is to remove toxins from your blood. If your liver is unable to do this due to cirrhosis, the levels of toxins in your blood can increase and lead to a condition called hepatic encephalopathy.

Symptoms of hepatic encephalopathy can include:

• agitation
• confusion
• disorientation
• muscle stiffness
• muscle tremors
• difficulty speaking
• in very serious cases, coma

Hepatic encephalopathy may require admission to hospital. In hospital, your body functions will be supported while medication is given to remove the toxins from the blood.

Hepatic encephalopathy is very uncommon in PBC and must be distinguished from concentration and memory problems linked to fatigue.

Vitamin deficiencies

Bile produced by the liver plays an important role in digestion, particularly in digesting fats. As the flow of bile out of the liver is obstructed in people with PBC, the condition may occasionally cause problems absorbing "fat-soluble" vitamins – including vitamins A, D, E and K.

The medication colestyramine, often used to reduce the itchiness associated with PBC, can also affect your body's ability to absorb some of these vitamins from food.

Significant deficiencies in these vitamins can cause problems such as:

• difficulty seeing in poor light
• reduced bone density (osteopenia)
• nerve damage (peripheral neuropathy)
• muscle weakness
• a tendency to bleed or bruise easily

If you're not getting enough of one or more of these vitamins, you doctor may recommend taking supplements.

Liver cancer

If you have PBC, the progressive damage to your liver slightly increases your risk of developing liver cancer.

Therefore, regular screening may be recommended to look for signs of cancer because the earlier the cancer is diagnosed, the greater the chance there is of curing it.

Screening will typically involve regular ultrasound scans and blood tests.

Read more about diagnosing liver cancer.