Definition:
Retroperitoneal fibrosis, also known as Ormond's disease, is a condition in which the fibrous tissue proliferates in the retroperitoneum, or the compartment of the body containing the renal tract, kidneys, aorta, and various other structures.
Diagnosis:
Retroperitoneal fibrosis can be diagnosed on the basis of a patient's family history as well as through radiologic testing. In some cases, diagnosis of the disease cannot be confirmed until further surgical exploration. Recent advances in research indicate that steroids can be used as an alternative to surgical ureterolysis.
Treatment:
Treatment for retroperitoneal fibrosis is commonly based on administering glucocorticoids, followed by DMARDs. In addition, SERM tamoxifen has also some proven efficacy in research trials, although further studies are needed to determine the mechanism of its action. Immunosuppressive drugs, including cyclophosphamide, azathioprine, and tamoxifen may also be used. However, if afflicted patients exhibit severe urinary tract obstruction, surgical intervention becomes almost always necessary.
Symptoms and Signs:
Common symptoms of retroperitoneal fibrosis include renal failure, deep vein thrombosis, lower back pain, hypertension, and a range of other obstructive symptoms.
Causes:
Retroperitoneal fibrosis is associated with a variety of immune-related conditions. The disease can also develop as a result of immunosuppression, leading to suspicion of an autoimmune etiology. A third of recorded retroperitoneal fibrosis cases are secondary to medication, malignancy, aortic aneurysm, or certain infections.
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