Retroperitoneal fibrosis

Last updated: July 29, 2023

Summarytoggle arrow icon

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 nonspecific 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%).

Epidemiologytoggle arrow icon

  • Prevalence
    • Rare (1 per 200,000–500,000 of the general population) [1]
    • Primary/idiopathic RPF: most common (70% of the cases) [2]
  • Peak age of incidence: 40–60 years [3]
  • Sex: > (2:1) [1]

Epidemiological data refers to the US, unless otherwise specified.

Etiologytoggle arrow icon

Primary/idiopathic retroperitoneal fibrosis

Secondary retroperitoneal fibrosis

Malignancies and exposure to methysergide are the most common causes of secondary RPF.

Pathophysiologytoggle arrow icon

Clinical featurestoggle arrow icon

Diagnosticstoggle arrow icon

Treatmenttoggle arrow icon

Medical therapy [4][9]

Decompression of obstructed retroperitoneal structures [11]

Prognosistoggle arrow icon

  • Prognosis of nonmalignancy 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) [12]

Referencestoggle arrow icon

  1. Engelsgjerd JS, LaGrange CA. Retroperitoneal Fibrosis. StatPearls. 2021.
  2. Cronin CG, Lohan DG, Blake MA, Roche C, Mccarthy P, Murphy JM. Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. 2008; 191 (2): p.423-431.doi: 10.2214/AJR.07.3629 . | Open in Read by QxMD
  3. Mzabi A, Kéchrid N, Alaya Z, et al. [Retroperitoneal fibrosis in adults: Diagnostic approach based on a retrospective multicenter study on 32 cases].. Prog Urol. 2019; 29 (2): p.76-85.doi: 10.1016/j.purol.2018.10.006 . | Open in Read by QxMD
  4. Urban ML, Palmisano A, Nicastro M, Corradi D, Buzio C, Vaglio A. Idiopathic and secondary forms of retroperitoneal fibrosis: a diagnostic approach. Rev Med Interne. 2015; 36 (1): p.15-21.doi: 10.1016/j.revmed.2014.10.008 . | Open in Read by QxMD
  5. Mann DA, Oakley F. Serotonin paracrine signaling in tissue fibrosis. Biochim Biophys Acta. 2013; 1832 (7): p.905-910.doi: 10.1016/j.bbadis.2012.09.009 . | Open in Read by QxMD
  6. Caiafa RO, Vinuesa AS, Izquierdo RS, Brufau BP, Ayuso colella JR, Molina CN. Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics. 2013; 33 (2): p.535-552.doi: 10.1148/rg.332125085 . | Open in Read by QxMD
  7. Tzou M, Gazeley DJ, Mason PJ. Retroperitoneal fibrosis. Vasc Med. 2014; 19 (5): p.407-414.doi: 10.1177/1358863X14546160 . | Open in Read by QxMD
  8. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006; 367 (9506): p.241-251.doi: 10.1016/S0140-6736(06)68035-5 . | Open in Read by QxMD
  9. Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet. 2011; 378 (9788): p.338-346.doi: 10.1016/S0140-6736(11)60934-3 . | Open in Read by QxMD
  10. Vaglio A, Corradi D, Manenti L, Ferretti S, Garini G, Buzio C. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med. 2003; 114 (6): p.454–462.doi: 10.1016/S0002-9343(03)00056-1 . | Open in Read by QxMD
  11. Tiptaft RC, Costello AJ, Paris AM, Blandy JP. The long-term follow-up of idiopathic retroperitoneal fibrosis. Br J Urol. 1982; 54 (6): p.620-624.
  12. Arrivé L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology. 1989; 172 (1): p.139-143.doi: 10.1148/radiology.172.1.2740497 . | Open in Read by QxMD

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