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Retroperitoneal fibrosis

Last updated: November 4, 2020

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Retroperitoneal fibrosis (RPF, Ormond's disease) is a rare disease of unknown etiology, characterized by inflammation and fibrosis of the retroperitoneum resulting in compression and encasement of the ureter, and/or the retroperitoneal blood vessels. RPF may be primary/idiopathic (most common) or secondary (e.g., drug-induced, inflammatory, iatrogenic). Patients often present with non-specific symptoms (e.g., fever, malaise, weight loss, flank pain, etc.). Bilateral ureteral obstruction, with subsequent hydronephrosis and obstructive nephropathy, is common. Diagnosis is often suspected in patients who present with bilateral hydronephrosis of unknown etiology. Contrast CT is the diagnostic test of choice and reveals a retroperitoneal mass encasing and obstructing the ureters and/or the aorta and IVC. Diagnosis is confirmed on CT-guided biopsy of the mass. High-dose glucocorticoids are the mainstay of treatment of primary RPF. Secondary RPF is managed by treating the underlying cause (stopping the offending drug, treating the infection, etc.). Symptomatic/severe obstruction of the retroperitoneal structures require treatment (ureteric stenting, ureterolysis, arterial stenting, etc.). Prognosis of non-malignancy-induced RPF is good, but recurrence rates are high (70%).

References:[1][2][3]

Epidemiological data refers to the US, unless otherwise specified.

Primary/idiopathic retroperitoneal fibrosis

Secondary retroperitoneal fibrosis

Malignancies and exposure to methysergide are the most common causes of secondary RPF!
References:[1][3][4][5]

References:[1][3]

References:[1][3][4][6][7][8][9]

References:[1][3][4][6][10]

Medical therapy

  • Primary RPF
  • Secondary RPF
    • Treatment of the underlying etiology: e.g., discontinue the causative drug, treat chronic infections, treatment of lymphoma, etc.
    • Oral high-dose glucocorticoids: Indicated in symptomatic/severe drug-induced cases of secondary RPF

Decompression of obstructed retroperitoneal structures

References:[1][4][8][11][12]

  • Prognosis of non-malignancy induced RPF is good, with symptomatic and clinical improvement obvious within a few weeks of initiating therapy.
  • High recurrence rates of idiopathic RPF (70%)
  • Poor prognosis of malignancy-induced RPF (∼ 6 months)

References:[1][11][13]

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