Guy's RPF Service
We established the first dedicated multidisciplinary RPF clinic in the UK at Guy's Hospital, London, in 2012
Our aim was - and remains - to improve the care of patients with RPF by bringing together specialist surgeons, nephrologists, pathologists, rheumatologists, radiologists, patients and research scientists
This clinic may now be the largest such service in the world. We receive weekly referrals of RPF patients and have seen over 750 patients from all over the world. We have performed complex RPF surgery (ureterolysis and ureteric reconstruction) on more than 300 patients, with the majority of these being performed using minimally invasive (robotic) keyhole surgery
We now run three multi-disciplinary clinics a month and run a monthly RPF multi-disciplinary team meeting to discuss particularly complex cases
We also offer a virtual review service for the convenience of clinicians and patients who are very far from London
We have established the first RPF biobank in the world and have won prizes for our research into RPF
Tim O'Brien
Tim O'Brien is a consultant urological surgeon at Guy's and St Thomas' Hospital. He has expertise in complex retroperitoneal surgery, kidney cancer and testis cancer. He is responsible for establishing the RPF service at Guy's and has given numerous talks on RPF all over the world
Archie Fernando
Archie Fernando is a consultant urological surgeon at Guy's and St Thomas' Hospital. She has expertise in both open and minimally-invasive (keyhole) surgery, and has introduced robotic ureterolysis in the UK for the treatment of ureteric obstruction from RPF. Her MSc thesis was on the use of PET scanning in RPF
James Pattison
James Pattison is a consultant nephrologist at Guy's and St Thomas' Hospital. He has expertise in managing patients with renal dysfunction requiring immunosuppressive medicines. He has been the principal investigator in many clinical trials studying new immunosuppressive agents in kidney transplantation
David D'Cruz
Professor David D'Cruz is a consultant rheumatologist at Guy's and St Thomas' Hospital and the National lead for IgG4-related disease. He has expertise in managing complex systemic diseases requiring immune-modulatory drugs
Catherine Horsfield
Catherine Horsfield is a consultant histopathologist at Guy's and St Thomas' Hospital with expertise in renal and urological pathology
Annafe De LaRosa
Annafe is the RPF specialist nurse and the point of contact for all our RPF patients
Zaid Viney and Christian Kelly-Morland are consultant radiologists with specialist interest in RPF imaging. They report all of our RPF scans and are part of the monthly RPF multi-disciplinary meetings where we review complex cases
What is it?
Retroperitoneal fibrosis (RPF) is a rare condition characterised by the presence of inflammation and fibrosis (scar tissue) in the retroperitoneal space. Anatomically, inflammation and fibrosis start surrounding the large blood vessel in the body, called the aorta, that delivers blood from your heart to the rest of your body, at the level just below your kidneys. The fibrosis progresses inferiorly toward the blood vessels that carry blood to your legs, and outwards towards the kidney(s) and surrounding structures, and ultimately could lead to kidney failure
What causes it?
RPF can be classified as primary (idiopathic) meaning that the cause is not known, or secondary based on the presence or absence of conditions known to be associated with RPF. These associated conditions include (1) leakage of tissue products from the wall of blood vessels / aneurysm; (2) drugs including methysergide, pergolide, bromocriptine, ergotomines, methyldopa, hydralazine and beta blockers; (3) external beam radiation that may be used to treat various forms of cancer; (4) recent abdominal or pelvic surgery; or recent abdominal/pelvic trauma; (5) IgG4-related disease; (6) infections such as tuberculosis, histoplasmosis, and actinomycosis. Depending on the case series 10-25 % of cases of RPF have had an identifiable associated condition. We think this is likely to be higher if patients have accurate diagnostics prior to commencing treatment e.g. steroid medication
How common is it?
RPF is a relatively uncommon disease. The estimated annual incidence from 1 per 200,000 to 500,000 population
Who gets RPF?
Anyone can get RPF and we have seen patients from aged 11 to 91 years of all ethnicities who have RPF
The symptoms and signs associated with RPF are often non-specific
Most patients are diagnosed when they present to hospital with severe back pain or renal failure because the kidney tubes have been blocked by the RPF
Pain, often accompanied by significant weight loss, constipation, and constitutional symptoms, is a common presenting symptom. Pain is present in up to 92% of cases; it is typically in the flank, back, scrotum, or lower abdomen and is dull, poorly localised, and constant
Other manifestations may include the following: fever; lower-extremity swelling; phlebitis (rash along the leg veins); deep venous thrombosis (blood clot)
Nausea, vomiting, anorexia, and malaise are uncommon
Raynaud's phenomenon (colour change of the fingers when exposed to cold conditions), ureteric colic (pain in the tummy or back that comes and goes in waves), hematuria (blood in the urine), claudication (pain in the legs or feet on walking that gets better when you stop), and urinary frequency occur rarely
Blood tests
In addition to carrying out routine blood tests looking at renal function and blood count, we check inflammatory status with CRP and ESR, immunoglobin subclass levels, and do additional blood testing for rarer conditions associated with RPF if appropriate
Imaging
We use CT scans for the initial detection / diagnosis of RPF. We then use PET scanning to determine whether the RPF is in an inflammatory or non-inflammatory phase. PET scanning also helps us to determine whether biopsy is required and to guide individual treatment strategy
We sometimes use kidney drainage scans (renograms) to check if the kidneys are blocked, and how much each kidney is contributing to overall kidney functioning
Where indicated we also use MRI scans, venograms, and arterial doppler scans
Biopsy
Excluding cancer is a very important part of the RPF diagnostic pathway. We a have conducted research that has proved PET scans very helpful in this regard. However, some patients still require biopsy which can be performed with a needle or surgically
Histopathology
We look for the presence of certain cells in the biopsy sample to work out to exclude cancer, diagnose RPF and help determine what subtype of RPF you have. If you have had a biopsy at your local hospital then Catherine Horsfield, our pathologist, will request the tissue and examine it herself
RPF Biobank
We have set up the first RPF biobank - tissue and blood samples are frozen for use in future RPF research. You will therefore be asked for you permission to biobank your tissue / blood. It does not change the tests or treatments that you will have but could potentially be life-changing for patients who develop RPF in the future
Our Treatment Goals
Right Diagnosis
Preservation of Renal Function
Freedom from Stents, Steroids and Pain
Right Diagnosis - please see section 'Our Diagnostics'
Preservation of Renal Function - Management of ureteric obstruction
If the inflammation and fibrosis surrounds the tube (ureter) leading from your kidney to your bladder and blocks the flow of urine this can cause the kidney to block and eventually fail. First and foremost this obstruction has to be relieved. This can be done either by placing a tube from your bladder though the ureter into the kidney (stent) or by placing a tube through your back into your kidney (nephrostomy)), allowing urine to drain through the tube
Sometimes these tubes are temporary but in many cases the kidneys may not be able to drain well without these tubes. In order to get your kidney tubes draining well without the need for artificial tubes you may require surgery called ureterolysis (see below)
Medication - Active RPF
There is evidence that this disease is mediated by the immune system. In the early phases of the disease, one can clearly see from biopsy specimens, cells that originate from the immune system and are responsible for the inflammation and likely subsequent fibrosis. A variety of agents, most commonly steroids have been used to suppress the immune system in an attempt to control the disease. We use an approach of high dose corticosteroids for a short period of time to shut down the inflammation whilst minimising the side effects. If the steroids are ineffective then other agents e.g. rituximab might be used. If longer term immunosuppression is required then steroid sparing agents are utilised early to minimise the debilitating side effects of long term steroids (e.g. bone thinning, diabetes, weight gain)
Freedom from Steroids - Inactive RPF
RPF has both an inflammatory (active) and non-inflammatory (inactive) phase. Our research has shown that steroid medication in patients who have inactive RPF is ineffective and that tailoring treatment depending on whether you have active or inactive RPF is beneficial
Freedom from Stents - Ureterolysis
In cases where the kidneys or not draining well with stents, where stents cause patients a lot of irritation or are problematic (e.g. require frequent changing), or where lifelong stents are indicated, an operation called ureterolysis can be performed. This is an operation to free the kidney tubes and move them away from the RPF tissue. Over 90% of patients on whom we have performed ureterolysis surgery are free of stents and nephrostomy tubes with excellent renal function. We have more experience of performing this surgery than any other centre in the world
We began the robotic (keyhole) ureterolysis program at Guy's in January 2016 in an effort to get patients' free of stents with a shorter hospital stay and less pain. We have now successfully performed >120 robotic ureterolysis operations. We continue to use open surgery in selected cases
Click here for patient information leaflet on ureterolysis
Freedom from Pain - Pain management
Pain in RPF can be multi-factorial. Some patients have improvement with steroid medication. Some have improvement following ureterolysis surgery and stent removal. In those patients who have persistent pain despite these treatments we offer nerve injections which have successfully relieved pain in several cases
Fatigue
Patients with RPF feel tired for a number of different reasons and we use a step-wise approach to identify and treat the different causes of fatigue in order to improve energy levels
Some patients have relapse / re-activation of RPF, involvement of other organs or recurrent blockage of kidney tubes. We therefore monitor our RPF patients long term using a combination of blood tests and/or scans. We have shown that certain subtypes of RPF require closer monitoring and so use an individualised monitoring schedule. Some patients require long term medication to control their RPF. However, our goal for patients is to get them free of medication where possible and living a normal life again
We have been working hard on behalf of our RPF patients to improve the understanding of RPF, its diagnosis and treatment. We have set up the first RPF biobank in the world and are freezing samples for future research. We have published and presented on RPF and won national and international awards for our research into RPF
PUBLICATIONS AND INTERNATIONAL PRESENTATIONS
A prospective study of robotic-assisted laparoscopic ureterolysis, including a comparison with open ureterolysis, in patients with ureteric obstruction from RPF Fernando A, Pattison J, De La Rosa A, O'Brien T Presentation at European Association of Urology 2018
Losing kidneys in RPF - a prospective study of kidney loss in patients with ureteric obstruction from RPF Fernando A, Horsfield C, D'Cruz D, O'Brien T Presentation at European Association of Urology 2018
18F-Fluorodeoxyglucose Positron Emission Tomography in the diagnosis, treatment stratification, and monitoring of patients with retroperitoneal fibrosis: a prospective clinical study. Fernando A, Pattison J, Horsfield C, D'Cruz D, Cook G, O'Brien T. Eur Urol. 2016
A lot of questions (and a few answers) in retroperitoneal fibrosis. Fernando A, Pattison J, Horsfield C, Bultitude M, D'cruz D, OBrien T. BJU Int. 2016 Jan;117(1):16-9. doi: 10.1111/bju.13061.9 http://onlinelibrary.wiley.com/doi/10.1111/bju.13061/abstract;jsessionid=ED8445092EEB3D90273963BA51CF0A33.f04t01
Understanding a new clinical entity - prospective study of patients with Immunoglobulin G4 related retroperitoneal fibrosis (IgG4-RPF) in a specialist service. Presentation at European Association of Urology 2017 and British Association of Urological Surgeons 2017
Ureterolysis in the treatment of ureteric obstruction from Retroperitoneal Fibrosis - treatment of first choice or last resort? Presentation at European Association of Urology 2017
Exploring the potential of FDG-PET in improving clinical decision-making in patients with Retroperitoneal Fibrosis. Presentation at European association of Urology and British Association of Urology 2016
We are always available to our patients for any support or help you may need. Please email annafe.larosa@gstt.nhs.uk
WHAT OUR PATIENTS SAY
“I can’t thank you enough for what you have done”
"I am so grateful that I found the Guy's RPF team"
"You have transformed my life"
"I am living without pain for the first time in 2 years"
"I never thought that I would be able to get rid of those horrible stents"
"What you do here is amazing and I would tell anyone with RPF that they have to come here"
PATIENT DAYS
We held the first RPF patient day in May 2016. We are planning another patient day in 2018. The date and details will be posted here shortly
PATIENT GROUPS
We held the first formal RPF MDT meeting in the UK on 23rd March 2018 and have continued to do this once a month
In 2020, due to the change in the nature of consulting and travel due to COVID-19, we began offering a virtual case review services where doctors from all over the UK and beyond could refer patients for a ‘virtual opinion’. We would review the imaging and case noted remotely and offer a recommendation so that the patient did not have to travel to London and could continue to safely be managed locally. Of course this is not always possible in a complex disease such as RPF where it can sometimes be essential that we see / examine the patient ourselves, perform specialist tests at Guy’s or offer treatment that is only available at Guy’s
We have been awarded prizes for our RPF research by the European Association of Urology
We run three RPF clinics a month at Guy's and a telephone clinic alongside our MDT to provide a better service for our increasing number of RPF patients
I have been diagnosed with RPF and want to be seen at the Guy's RPF Service. What should I do?
Email: masud.miah@gstt.nhs.uk; or
Telephone: 0207 188 7338
Where is the Guy's RPF Service located?
Guy's Hospital, Urology Centre, 1st Floor, Southwark Wing, Great Maze Pond, London, SE1 9RT
The closest station to the hospital is London Bridge Station
You can find a map showing the location of the clinic within the hospital here
What are the clinic times?
Clinic times are 1.30pm to 5.00pm on the 1st, 2nd and 3rd Mondays of every month. We used to have only 2 clinics a month but have increased this to provide a better service for our increasing number of RPF patients
