Polycystic Liver Disease
Key points
- Polycystic Liver Disease (PLD) is a rare disease you inherit
- It can happen by itself, or you might also have polycystic kidney disease
- Women are more likely to have symptoms
- If the liver becomes very large and causes symptoms, a liver transplant may be required
What is PLD?
It is estimated that around 5 to 10 in every 100 people in the world have liver cysts (fluid filled sacs in the liver). Polycystic liver disease is a rare genetic condition where you have multiple (more than 10) cysts.
Most cysts will be small and won’t cause any symptoms. But over time, some people will start to have symptoms as the cysts get bigger. The cysts gradually to take up more room in your abdomen (belly), making it difficult to eat, exercise and move around as easily as you normally would. The impact on quality of life is often a reason for people to be referred for liver transplant.
A rare complication of PLD can be a ruptured (burst) cyst. This could cause severe abdominal pain and lead to infection or internal bleeding. If you experience this sort of pain suddenly, sometimes also with a fever, you would need to attend an emergency department immediately.
What are the symptoms of PLD?
PLD often has no symptoms. If you have a lot of cysts, or they are getting bigger, you may experience the following symptoms:
- Difficulty bending
- Abdominal pain and back pain
- Swollen abdomen (tummy)
- Feeling very full, and unable to eat usual amounts
- Feeling breathless
- Being able to feel lumps on your belly
- Fever and acute pain if a cyst has burst
If you are experiencing any of these symptoms, you should see your doctor for further testing.
How is PLD diagnosed?
PLD is often found in people who are having an abdominal (belly) scan for another reason. These tests can include ultrasound, CT scans or an MRI scan. If liver cysts are seen during one of these scans, your doctor will then take a medical history to see if anyone else in your family has had this condition. They will also do a physical examination to see if they can feel any cysts, and may do blood tests, although liver function blood tests will usually remain normal.
You will receive a diagnosis of PLD if you have more than 10 cysts in your liver. If you have a family history of PLD, it is diagnosed if you have more than 4 cysts in your liver.
Why did I get PLD?
PLD is an inherited condition, meaning it can run in the family. It is an autosomal dominant condition. This means 1 in 2 people with an affected parent will inherit the gene causing PLD.
Most people with PLD never know they have it because it doesn’t cause symptoms. But even if one of your parents had the condition and had no symptoms, you may have large cysts that cause symptoms.
It’s thought that the female hormone oestrogen is involved in PLD, and more women than men will develop severe symptoms from the disease.
Many people with PLD also have polycystic kidney disease (PKD), which can cause high blood pressure and progressive kidney failure leading to need for dialysis or kidney transplantation.
How is PLD treated?
Most people will not need treatment. If you start to get symptoms, you will be referred to a specialist to work out the best treatment for you. This could include:
- Stopping the contraceptive pill, or other oestrogen treatments
- Antibiotics if you have infected fluid in the cysts
- Lanreotide or Octreotide injections – this is a medication that is given as a small injection into your tummy or buttock every 4 weeks. Some studies have shown that it reduces the volume of cysts
- Draining – this is a surgical procedure where the fluid is drained out of very large cysts
- Resection – this is surgery to remove a section of liver containing multiple cysts
- Liver transplant – if this is recommended, you will be referred to a specialist liver transplant centre. Transplant is usually recommended if your quality of life is severely affected, and other treatments have not been successful or are not recommended. For people with severe polycystic kidney disease, a combined liver and kidney transplant may be recommended.
Living with PLD
Most people with PLD will have no symptoms and may not even know they have the disease. If you are known to have it, you may be asked to stop all medications containing oestrogen, including hormone replacement therapy and the contraceptive pill. It is important you discuss this with your doctor.
- If you are experiencing symptoms, you may find it hard to do everyday tasks. You may feel very tired. You may have to stop playing contact sports because of the risk of a cyst bursting if you get hit in the tummy.
- You could get indigestion (heart burn) because the liver is so big it is pressing on your stomach. If this is happening, try to have regular small meals rather than 3 big meals each day.
- If your tummy is very large, you may find it is difficult to get comfortable in bed, or you get short of breath when you lie flat. It will take a bit of trial and error to find a position that is most comfortable for you.
- It can be difficult if you are a young woman with PLD, as people may assume you are pregnant if you have a very swollen tummy. If you are struggling emotionally, there are services that can assist.
- If you are struggling to eat, you may be referred to a dietitian who will help you to manage your diet and offer supplements if necessary.
- It is important to remember that only 10 to 15 in every 100 people with PLD will develop obvious symptoms.
More information
British Liver Trust. Polycystic liver disease.
What next?
Call 1800 841 118 to speak to a liver nurse
Read about living well
References
van Aerts RMM, Kievit W, D’Agnolo HMA et al. Lanreotide Reduces Liver Growth In Patients With Autosomal Dominant Polycystic Liver and Kidney Disease. Gastroenterology. 2019 Aug;157(2):481-491.e7. doi: 10.1053/j.gastro.2019.04.018.
Cleveland Clinic. Liver cysts.
Reviewed April 2025