Find out about the different types of pain ADPKD can cause, and how this pain can be treated
Chronic pain (defined as persistent or longer-term pain)is common in people with autosomal dominant polycystic kidney disease (ADPKD). About 6 in 10 people who have been diagnosed with the condition have chronic pain. An enlarged kidney or liver pressing on other organs or tissues is often the likely cause of this pain.
Urinary tract infections (UTIs) are common among people with ADPKD, and are a particular problem for women. These infections should always be taken seriously and fully investigated, because frequent UTIs may worsen kidney function in some people with ADPKD.
Around 4-8 in 100 people with ADPKD have a small aneurysm: a ‘ballooning out’ of a blood vessel due to weakness in the vessel wall. Aneurysms may occur in the blood vessels of the brain (when they are called intracranial aneurysms, ICA or ‘berry aneurysms’).
Find out how cysts and other problems can develop in the livers of people with autosomal dominant polycystic kidney disease (ADPKD). We explain causes, symptoms, possible complications and treatment.
What is polycystic liver disease?
If you have ADPKD, you’re likely to develop lots of cysts in organs other than your kidneys, most commonly in your liver. This is known as polycystic liver disease (PLD).
Recording of the Polycystic Liver Disease (PLD) webinar organised by the PKD Charity, 1 February 2021.
People without ADPKD can get PLD too. About 1 in 500 people carry genes that can cause PLD. Not all of these people develop symptoms or are diagnosed with PLD.
This factsheet is for people with ADPKD who also have PLD. If you have autosomal recessive polycystic kidney disease (ARPKD), please see our factsheet ‘ARPKD: a guide for parents’ for information on how this disease can affect the liver.
The speed at which liver cysts develop in people with ADPKD varies from person to person, but generally the number and size of cysts gradually increases from early adulthood. About 9 out of every 10 people with ADPKD develop liver cysts by their mid-thirties. Some others might never be diagnosed with PLD.
ADPKD can cause other changes to the liver and digestive system too. These include:
an enlarged liver or bile duct (a tube that carries fluid to help digestion from the liver to the bowel)
hernias (where part of the intestines comes through the abdominal muscle)
Most people with PLD don’t have a very enlarged liver, but in severe cases the liver can more than triple in size. This might cause pain if the liver pushes against other organs.
PLD is more common in women than in men with ADPKD. In women, more cysts occur at a younger age and these tend to be larger. This is thought to be because the female hormone oestrogen is involved in the development and growth of liver cysts.
Liver cysts develop in the tubes of the liver (bile ducts or liver tubules) rather than in the specialised tissue that the liver needs to do its job. This means that liver cysts rarely cause liver failure, even if they make your liver larger.
Fortunately, most people with ADPKD and liver cysts don’t have any symptoms. Despite the cysts, your liver is likely to still function well. It is unlikely you’ll need surgery to reduce the size of your liver. It is even less likely that you’ll need a liver transplant.
Causes of liver cysts
In people with ADPKD, experts think liver cysts are probably caused by the same genes that cause kidney cysts.
The hormone oestrogen appears to increase the development and growth of liver cysts. This hormone is found in higher levels in women than men. Women who have been pregnant are likely to develop liver cysts earlier and have worse liver cysts than women who have never been pregnant. Women who have used oestrogen-containing contraception or hormone replacement therapy (for the menopause) might get liver cysts sooner or worse cysts.
Symptoms of liver cysts
Most people with PLD will never have any symptoms. Most people with ADPKD only find out they have liver cysts because these are spotted on a scan. Scans make images of the inside of your body.
You may start getting symptoms as you get more liver cysts and they grow bigger, pushing on other organs or blood vessels. Your symptoms may include:
feeling bloated and having a larger abdomen (stomach) – this can be caused by an enlarged liver and by fluid collecting in your abdomen (ascites)
feeling full early on in a meal
heartburn (acid reflux)
abdominal (stomach) pain
Occasionally, an enlarged liver might cause shoulder pain (this is known as referred pain).
If you have any of these symptoms, let your GP or kidney team know. They can refer you to a liver specialist (hepatologist) for assessment if they think your symptoms might be liver related.
Some people with PLD tell us that their enlarged abdomen can be troublesome. For example, it can make it difficult for them to bend over and be uncomfortable to sleep on their side or stomach. People can find having a large abdomen upsetting, affecting their confidence or how they feel about themselves.
It is unlikely that your liver cysts will cause serious problems that need treatment. Only about 1 in every 20 people with PLD (5%) gets complications. However, these can include:
your cysts may bleed or burst, causing sudden pain
your cysts may become infected, causing pain and fever
If large veins in the liver or bile duct become squashed by a cyst, this can cause problems such as:
a build-up of waste that the liver usually clears, leading to yellow skin (jaundice)
hardening of the liver (known as fibrosis)
Other liver and gastrointestinal symptoms of ADPKD
If you have ADPKD, you might get other problems with your liver or gastrointestinal tract (stomach and bowel) that are not caused by liver cysts. These include:
diverticular disease (pouches forming in the colon wall), which is very common in ADPKD patients with kidney failure
a hernia, which is when tissue inside your abdomen pushes through a muscle or tissue wall, causing a bulge (about half of ADPKD patients get this)
cysts in your pancreas (up to about 1 in every 3 patients get these), or less commonly, in your spleen
a slightly increased risk of gall stones or gall bladder infections (the gallbladder is a pouch that stores bile for digestion)
Diagnosing liver cysts
Your doctor or hepatologist will check your and your relatives’ medical history, and examine you. Liver cysts are usually diagnosed using ultrasound, computed tomography (CT) or magnetic resonance imaging (MRI) to check the size and position of your liver cysts. An ultrasound uses soundwaves to see inside your body, while a CT scan uses X-rays, and an MRI uses magnets and radiowaves.
You’ll be diagnosed with PLD if you have 20 or more liver cysts.
You may also have blood tests to check for signs of liver problems, particularly if you have symptoms of a possible infection.
Monitoring your liver health
If you’re diagnosed with PLD, the ongoing care you receive will be adapted to your symptoms and needs. Your doctor or hepatologist will monitor your general liver health as well as your kidneys.
Follow up plans can vary. If you have PLD that is not causing complications, you may have a check-up about once a year. In follow up appointments, your hepatologist will ask about your symptoms and do a blood test to check your liver function. You may also have imaging of your liver every 1—3 years.
You’re very unlikely to need treatment for your liver cysts unless they’re causing symptoms that are affecting your life.
Treating liver cysts
If you do need treatment, you’ll be referred to specialists in treating liver problems, such as:
A liver specialist (hepatologist)
A liver surgeon
A specialist in using imaging in the treatment of disease (an interventional radiologist)
A kidney specialist (nephrologist).
We explain some treatments below.
If one of your liver cysts becomes infected, you’ll be given antibiotics to clear the infection. These may need to be started intravenously, meaning you’ll receive them via a drip in hospital. Cyst infections can be hard to treat, so you might need quite a long course of antibiotics (e.g. 6 weeks).
Procedures to treat liver cysts
Procedures, minor and major surgery for PLD are listed in the table below. The risks of procedures or surgery vary depending on the type of procedure and your general health. Your surgeon will talk you through any risks you should consider as well as the likely benefits. He or she will also explain what to expect, how to prepare and your recovery period.
How well the treatment works will depend in part on the size and number of cysts you have and the type of procedure.
Having surgery on your liver generally has greater risks than simpler procedures. So, if simpler procedures are likely to work, your doctor will recommend these over surgery. He or she will only recommend surgery for you if the benefits are likely to be greater than the risks.
Drainage of large cysts (aspiration)
This can be done using either a tube inserted through the skin using local anaesthetic or an operation under general anaesthetic. Local anaesthetic numbs an area of your body so you can’t feel pain, whereas general anaesthetic makes you unconscious during the procedure.
After draining, a large cyst is sometimes treated with a chemical such as ethanol (a type of alcohol) to kill the cells lining it. This is called sclerotherapy and help to stops the cyst filling with fluid again.
This procedure treats blood vessels supplying liver cysts to stop the cyst’s blood supply. For this procedure, a thin tube is inserted through the skin into one of your blood vessels. The tube is passed through your blood vessels to the small one that that feeds the cyst in your liver. That blood vessel is blocked.
Key-hole surgery (laparoscopy)
This type of surgery is used to open a cyst, remove the fluid, and take out the cells lining it (called cyst fenestration or deroofing).
Partial liver resection
This type of surgery can be used to remove the part of the liver most affected by cysts.
The vast majority of people with PLD do not need a liver transplant. The operation is sometimes done at the same time as a kidney transplant, if a person needs both organs transplanting.
If drainage or fenestration doesn’t work or is not recommended for you, an alternative option might be treatment with drugs called somatostatin analogues (e.g. octreotide and lanreotide). These drugs have been shown in trials to stop the liver increasing in size in some (but not all) people with ADPKD.
Although somatostatin analogues are approved for the treatment of some other conditions, they’re not yet approved in the UK for the treatment of ADPKD. Your doctor may be able to prescribe them, if he or she believes the benefits outweigh the risks for you. These drugs might also be available as part of a clinical trial.
Somatostatin analogues can have side effects including diarrhoea or constipation, abdominal pain, vomiting, flatulence, and gall stones.
At the moment, experts think that a treatment for ADPKD called tolvaptan (Jinarc®) does not reduce the size of liver cysts (although it can help to slow growth of kidney cysts. More research is needed before they can be sure.
If your liver cysts are causing pain, your doctor will recommend pain medications to help. You can find out more about managing pain on our page Pain from ADPKD.
Preventing liver cysts
It is not possible to stop cysts forming in the liver. If you’re a woman, you may want to consider avoiding oestrogen-containing contraception (e.g. the ‘Pill’) or hormone replacement therapy. Although oestrogen is linked to cyst growth, there is no hard proof that avoiding these medications would slow cyst progression, so discuss possible risks and benefits with your doctor.
Diet and lifestyle
There is not any general guidance available on diet or lifestyle for people with PLD. Our factsheet on diet and lifestyle has for tips for people with ADPKD (these tips relate to kidney function and general health).
Written by Hannah Bridges, PhD, Independent Medical Writer, HB Health Comms Limited. Expert review by Dr Ahmed Elsharkawy, Consultant Hepatologist, University Hospitals Birmingham NHS Foundation Trust.
With thanks to all those affected by ADPKD who contributed to this publication.
The information on this page is under review by the PKD Charity using the Information Standard process.
PKD Charity Helpline:The PKD Charity Helpline offers confidential support and information to anyone affected by PKD, including family, friends, carers, newly diagnosed or those who have lived with the condition for many years.
Disclaimer: This information is primarily for people in the UK. We have made every effort to ensure that the information we provide is correct and up to date. However, it is not a substitute for professional medical advice or a medical examination. We do not promote or recommend any treatment. We do not accept liability for any errors or omissions. Medical information, the law and government regulations change rapidly, so always consult your GP, pharmacist or other medical professional if you have any concerns or before starting any new treatment.
A diverticulum (plural: diverticula) is a small pouch that sticks out from the side of the colon (large bowel). Diverticula can affect anyone, especially older people. However, they seem to be more common in people with ADPKD than in people without kidney problems.