Retroperitoneal fibrosis

Retroperitoneal fibrosis or Ormond's disease is a disease featuring the proliferation of fibrous tissue in the retroperitoneum, the compartiment of the body containing the kidneys, aorta, renal tractand various other structures. It may present with lower back pain, renal failure, hypertension, deep vein thrombosisand other obstructive symptoms.

Its association with various immune-related conditions and response to immunosuppressionhave led to speculations as to the autoimmuneetiology of idiopathic RPF. One-third of the cases are secondary to malignancy, medication (methysergide, hydralazine, beta blockers), aortic aneurysmor certain infections.

In the absence of severe urinary tract obstruction (which generally requires surgerywith omental wrapping) treatment is generally with glucocorticoidsinitially, followed by DMARDseither as steroid-sparing agents or if refractory on steroids. The SERM tamoxifenhas shown to improve the condition in various small trials, although the exact mechanism of its action remains unclear.

References

• van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002;60:231-42. PMID 12365466.
• Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948;59:1072-79.
• Ormond JK. Idiopathic retroperitoneal fibrosis: a discussion of the etiology. J Urol 1965;94:385-90.

External link

• Emedicine
• Whonamedit- Dr John Kelso Ormond

This article is licensed under the GNU Free Documentation License.
It uses material from the http://en.wikipedia.org/wiki/Retroperitoneal+fibrosis Wikipedia article Retroperitoneal fibrosis.