Retroperitoneal fibrosis

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

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Causes

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

Treatment

In the absence of severe urinary tract obstruction (which generally requires surgery with omental wrapping) treatment is generally with glucocorticoids initially, followed by DMARDs either as steroid-sparing agents or if refractory on steroids. The SERM tamoxifen has 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.