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 tract and various other structures. more...

It may present with lower back pain, renal failure, hypertension, deep vein thrombosis and other obstructive symptoms.

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 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.

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