Retroperitoneal Fibrosis

Discussion in 'Fibromyalgia and ME & Chronic Fatigue Syndrome' started by MEH3400, Aug 8, 2012.

  1. MEH3400

    MEH3400 New Member

    I have yet to talk to anyone who has this. It is a very rare autoimmune disease. I am seeing a Rheumatologist and I am the second case he has treated in his entire career. Early on I tried to find another doctor to get a second opinion but I was turned away by every doctor I called. Please reply if you have it or know someone who does. I have been doing research and searching the internet on this awful disease and learning about it with my doctor.
  2. kris88

    kris88 New Member

    I also have retroperitoneal fibrosis as well as IgG4-related sclerosing blood disease, which is associated with RPF. I had surgery in April and have been under the care of an oncologist for both conditions since May.
  3. skeptik2

    skeptik2 New Member

    What is this? I've never heard that name before...

    skeptik2
  4. kris88

    kris88 New Member

    Retroperitoneal Fibrosis is a rare medical condition which causes plaque of fibrous tissues in the region at the back of peritoneum, next to abdominal aorta. Due to the masses formed, the ureters become enveloped which makes it blocked. This leads to a series of kidney problems, which include stoppage of urine and even kidney failure in severe cases. The abdominal aorta is often also enveloped in the fibrous tissue, as well. RPF is regarded as a form of IgG4-related disease, a blood antibody illness that can be detected on tissue samples and often at elevated levels in the bloodstream, in which inflammatory cells cause fibrosis, the deposition of connective tissue, in one or more organs. It's the IgG4 that creates the fibrous tissues throughout the body. While RPF is most commonly found on the ureters and abdominal aorta (as in my case), I also have fibrous nodes in my chest, apparently also caused by the IgG4 blood condition. Generally, neither illness is fatal, as long as they are diagnosed and treated. But since it's so rare, and because the symptoms often are attributed to other, more common ailments, it's often not diagnosed until the patient has progressed to kidney failure or other life threatening stages. RPF and IgG4 can be put into remission, but cannot be cured. There is also a significant incidence of recurrence, so patients considered in remission must be closely monitored and have CT scans frequently (some say at least twice a year) for the rest of their lives. Since the illness is so rare, there isn't much knowledge about it or the treatment of it. However, the prevailing treatment is with high doses of corticosteroids, primarily Prednisone, a drug that really kicks your butt. Some success has also been seen with certain chemotherapy drugs.