Talk:Retroperitoneal fibrosis

How

How was I supposed to know that the Lancet were going to publish a seminar on this 2 days after its creation? I'll update the page as necessary. JFW | T@lk 00:08, 25 January 2006 (UTC)

adding sections to Retroperitoneal Fibrosis (RF sometimes referred to as IRPF)

1st suggested addition: Support Groups There are a few on-line support groups for those with Retroperitoneal Fibrosis and/or their caregivers. The most active are:

https://groups.io/g/RetroperitonealFibrosis with about 300 members. This was a Yahoo group, but changed hosts (to groups.io) when Yahoo closed all groups. This group has the most extensive collection of articles, papers, videos, links, etc available anywhere on the web. There are also archives of messages posted since 2001 when the original RF support group began. Join the group to access these resources.

Another RF support group is a Face Book Page:

https://www.facebook.com/groups/60126946140/ with 750 members at start of 2021 (many are also members of above groups.io group). This group is found by joining Facebook and searching on Retroperitoneal Fibrosis or using the link posted above.

the following sites do not seem to be appropriate or active. 1st seems to be Put Morgan's page, second seems dead.:

http://www.facebook.com/#!/groups/229976760369942/ — Preceding unsigned comment added by 98.23.50.223 (talk) 07:17, 8 March 2012 (UTC)

http://retroperitonealfibrosis.com/

2nd suggested addition:

What's next for the patient?

Retroperitoneal Fibrosis (RF) is not life threatening if treated correctly/competently, but it is chronic. It will never go away and it should be monitored forever (blood work & scans), but the symptoms usually abate considerably with proper care.

However, just exactly what is "proper" care for a patient with Retroperitoneal Fibrosis has not been proven by any published trial results. (There has only been one randomized, double blind study related to RF and that was to compare prednisone vs tamoxifen. That study showed tamoxifen to be less effective than prednisone at reducing the RF mass.) However, there are a few basically standard protocols which seem to have been accepted by most practitioners with experience in the treatment of Retroperitoneal Fibrosis which will be discussed below under treatments.

Retroperitoneal Fibrosis is an extremely rare disease and most Doctors only hear about it in Med school, and never see an actual patient. Therefore it is up to the RF patient and/or their caregivers to be proactive in making sure that the proper care is given and that attention is given so as to minimize damage to kidney function.

As a patient, what's going to happen now.

You have RF. You will always have RF. It will NEVER go away. It may go into remission, but can always flare up again.

But patients can manage RF once the present symptoms are alleviated or at least reduced to a livable level. And some patients seem to have complete remissions, usually as a result of medication, very rarely without medication (spontaneous remission).

Most important is for you to find an RF aware Dr, usually a Rheumatologist to start, who will be your primary Dr for the RF treatment. The Rheumatologist will refer you to other specialists as necessary. It is possible for patients who can not avail themselves of an RF aware Dr to have their Dr consult with one of the expert RF aware centers. (Guys in London, Mayo in USA are two of these. Others are available by asking on one of the support groups mentioned above.)

Management means regular tests:

Blood:

     Creatine to make sure that both ureters are not being constricted, 
     BUN, ESR, eGFR, & CRP to check for changes in internal inflammation. This would be an early warning that worse is on its way. And by early, sometimes that warning can be months ahead of other symptoms.

Scans:

     CT or MRI to follow changes in the Retroperitoneal Fibrosis mass which has been described as tough as wood, scar tissue, hardened, etc. MRIs are much safer for you in the long run as they do not involve X-Rays (radiation exposure), but MRIs may be a bit harder to interpret, but also may include much more detail than a CT scan. Scans for RF usually include contrast to highlight the RF mass.
     Renal scans of one kind or another (ultrasound or mag 3 are two.) depending on what the Retroperitoneal Fibrosis decides to attack next.

Possible treatments

Will be up to your Dr to explain. Your Dr should be RF aware. (Both of the support sites mentioned above have a worldwide list of known RF aware Drs, but there are not very many.) Steroids, imuran, cellcept, or rituximab infusion - which seems to work best on IgG4+RF - and/or surgery (stents are considered surgery) are options. Usual treatment is preds at high dose (average is 60 mg/day) for about three months followed by slowly weaning/withdrawing from preds and gradually increasing one of the three most often used and most successful follow-on meds (imuran, cellcept, rituximab). These meds are used for many months, often years in an effort, often extremely successful, to reduce the RF mass from around the aorta and ureters.

If ureterolysis surgery is done it is very important that a complete/entire omental wrapping of the ureter is used to protect against Retroperitoneal fibrotic tissue re-growing & again attaching & encasing ureters (& other organs/structures) particularly where less than a full wrap was undertaken. If needed because there is not adequate omentum be aware of alternate "wrap material" eg. perinephric fat or other fat from within the abdomen.

If treatment doesn't work ask your Dr to check for IgG4+RF which may require an surgical biopsy (needle biopsy usually does not capture sufficient tissue for biopsy) if blood test for IgG4 is inconclusive (60-70 % of IgG4+RF does not show positive in blood work). IgG4+RF usually responds to rituximab infusions.

and/or ask for tests for ECD (Erdheim-Chester Dosease) which is sometime mistaken for RF. Treatment is similar, but different. More information on ECD is at https://erdheim-chester.org/care-centers/

32.210.12.47 (talk) 23:38, 6 February 2021 (UTC) suggested by Putnam Morgan, Connecticut, USA 2.6.2021

Pmorgan7 (talk) 02:37, 9 April 2010 (UTC)

IgG4

doi:10.1097/BOR.0b013e3283412f7c IgG4 deposition seems to be an important mechanism. JFW | T@lk 13:46, 20 June 2011 (UTC)

endometriosis

It is recognized that endometriosis can lead to retroperitoneal fibrosis. 68.199.114.39 (talk) 20:09, 10 April 2024 (UTC)