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, kidney failure, hypertension, deep vein thrombosis, and other obstructive symptoms. It is named after John Kelso Ormond, who rediscovered the condition in 1948.^[1][2][3]

Causes

Its association with various immune-related conditions and response to immunosuppression have led to speculation regarding an autoimmune etiology of idiopathic RPF.^[4][5] One-third of the cases are secondary to malignancy, medication (methysergide, hydralazine, beta blockers), aortic aneurysm, or certain infections.

Diagnosis

The diagnosis of retroperitoneal fibrosis cannot be made on the basis of results of laboratory studies. CT is the best diagnostic modality: a confluent mass surrounding the aorta can be seen on a CT scan. Although biopsy is not usually recommended, it is appropriate when malignancy or infection is suspected. Biopsy should also be done if the location of fibrosis is atypical or if there is an inadequate response to initial treatment.^[5]

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.^[6] The SERM tamoxifen has shown to improve the condition in various small trials, although the exact mechanism of its action remains unclear. Associations include:

• Riedel's thyroiditis
• previous radiotherapy
• sarcoidosis
• inflammatory abdominal aortic aneurysm
• drugs

References