What is retroperitoneal fibrosis?
Retroperitoneal fibrosis is a rare condition that causes scar tissue to develop in the blood vessels and ureters (the tubes connecting the kidneys and bladder). This can block the flow of urine causing back pressure that can damage the kidneys.
The retroperitoneum is the area of the body behind the stomach and intestines and in front of the backbone where the ureters run through.
Fibrosis is a type of internal scarring.
Retroperitoneal fibrosis affects around 1 in 100,000 people in the UK. It affects twice as many men as women. It can occur at any age, although it is usually first diagnosed around the age of 60 and is extremely rare in children.
What are the signs and symptoms of retroperitoneal fibrosis?
Some people with retroperitoneal fibrosis do not have any symptoms. For those who do, common symptoms include:
- pain in the abdomen, lower back or groin
- pain in the legs, which is worse when walking
- swelling of one leg due to blocked blood supply
- jaundice (yellowing of the skin and the whites of eyes)
- fevers and/or night sweats
- loss of appetite
- weight loss
- lack of energy.
These symptoms may come and go. There can be times when the condition becomes inactive, but it may then flare up again at a later date.
What causes retroperitoneal fibrosis?
The exact cause of retroperitoneal fibrosis is not yet known as there appears to be a variety of possible causes including:
- an overactive immune system
- use of a migraine medication called methysergide
- blood vessel disease caused by an unhealthy lifestyle, especially heavy smoking
- exposure to asbestos
- some forms of cancer
- other conditions such as vasculitis.
How is retroperitoneal fibrosis diagnosed?
Retroperitoneal fibrosis is usually diagnosed in adults by an ultrasound scan of the kidneys. A computed tomography (CT) or magnetic resonance imaging (MRI) scan may also be used to give a more detailed view.
Blood tests will be used to check kidney function.
A kidney biopsy may be recommended if the initial cause of the problem is unclear, although this may just show general scarring rather than anything specific to retroperitoneal fibrosis.
Does retroperitoneal fibrosis affect other parts of the body?
Retroperitoneal fibrosis can affect lots of area of the body, as detailed in the symptoms above.
Does retroperitoneal fibrosis run in families?
There is some recent research that retroperitoneal fibrosis may run in families, but the precise details of any inheritance patterns are still being investigated.
How is retroperitoneal fibrosis treated?
There is currently no direct cure for retroperitoneal fibrosis, so treatment aims to:
- protect kidney function
- reduce pain
- sow down the scarring process.
Different treatments work for different people, depending on the cause of their retroperitoneal fibrosis.
If it is caused by an overactive immune system, steroids or immunosuppressive medications may be prescribed to dampen the immune system and reduce scarring.
If the retroperitoneal fibrosis is linked to blood vessel disease, people may be advised to follow a healthier lifestyle. This can include:
- taking regular exercise
- maintaining a healthy body weight
- avoiding smoking
- eating a balanced diet.
GPs and specialist kidney dietitians can help with this.
If retroperitoneal fibrosis causes extensive scarring to the ureters, the flow of urine may become blocked. Surgery will then be needed. A thin tube called a stent is inserted into the ureter to keep it open so that the urine can flow easily.
This procedure is done as a day case under either a general anaesthetic (when you are put to sleep) or a spinal anaesthetic (where you are awake but cannot feel anything below the waist). The stents usually need to be replaced every few months as they can get blocked.
If stents are not effective, an operation called ureterolysis may be needed to free the ureter from the fibrous tissue and reduce the chance or future blockages.
Most people with retroperitoneal fibrosis can live normal lives with regular monitoring. It is rare for dialysis or transplantation to be needed.
Where can I get more information or support about retroperitoneal fibrosis?
We are not aware of any UK-based support groups for retroperitoneal fibrosis. If you know of any retroperitoneal fibrosis patient groups, please let us know so we can share their details.
Publication date: 11/2023
Review date: 11/2026
This resource was produced according to PIF TICK standards. PIF TICK is the UK’s only assessed quality mark for print and online health and care information. Kidney Care UK is PIF TICK accredited.