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School of Medicine
What is it?
Retroperitoneal fibrosis (RPF) is a rare condition characterized by the presence of inflammation and fibrosis in the retroperitoneal space. The disease process typically begins with clinical symptoms of flank pain, and unexplained weight loss. Anatomically, inflammation and fibrosis start surrounding the large artery, called the aorta, that delivers blood from your heart to the rest of your body at the level just below your kidneys. And progresses inferiorly toward the arteries that carry blood to your legs, and outwards towards the kidney(s) and surrounding structures, ultimately leading 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 associative conditions include (1) infections such as tuberculosis, histoplasmosis, and actinomycosis; (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. Depending on the case series 10-25 % of cases of RPF have had an identifiable associated condition.
How is it diagnosed ?
The first step in establishing a diagnosis is to obtain an x-ray. This may be in the form of a CAT scan or MRI. If inflammation is seen surrounding the Aorta or your kidney then you will need a biopsy of this area to accurately diagnose the x-ray findings as retroperitoneal fibrosis. Your doctor will also obtain a blood test to check your kidney function and look for the presence of anemia (low blood count), and for markers of inflammation in the blood.
How is it treated ?
The best way to treat retroperitoneal fibrosis is not known. 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 fail. First and foremost this obstruction has to be relieved by placing a tube through your back into your kidney, allowing urine to drain through the tube. Alternatively, the obstruction can be relieved by placing a tube from your bladder though the ureter into the kidney. Surgery can also be performed where the affected ureter is moved away from the inflammation.
Although the procedures mentioned above may relieve the obstruction, they do not stop the ongoing inflammation.
There is a growing body of 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. Therefore multiple different centers have used a variety of agents to suppress the immune system in an attempt to control the disease. In most patients both immunosuppression and temporary relief of obstruction is required. Our center has used an approach of low dose corticosteroids for a short period of time combined with mycophenolate mofetil, another drug used to suppress the immune system, with excellent results.
I have been diagnosed with retroperitoneal fibrosis and want to be seen at Johns Hopkins what should I do?
Patients interested in being evaluated at Johns Hopkins should call 410-955-5268 (select option #4) and ask to speak with Rose. You should obtain copies of your medical records. These would include copies of doctor’s visits, and hospitalizations. If you have had an MRI or CAT scan you should obtain copies of those studies. This may be in form of actual films or on a CD. If you have had a biopsy, you should obtain the actual biopsy slides from the hospital. All of the above should be forwarded to:
Dr. Paul J. Scheel, Jr., M.D.
Director, Division of Nephrology
Johns Hopkins University
1830 East Monument Street, Suite 416
Baltimore, Maryland 21287