• Clinical science

Retroperitoneal fibrosis (Ormond disease)

Abstract

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%).

Epidemiology

  • 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)[3]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

Primary/idiopathic retroperitoneal fibrosis

  • Immune reaction to antigens within aortic atherosclerotic plaques
  • Systemic autoimmune disease: RPF may be a systemic autoimmune disease of large arteries → periaortic inflammationinflammation and fibrosis in the periaortic region
  • IgG4-related disease (immunoglobulin G4 related disease): characterized by an infiltration of various organs by IgG4-bearing plasma cells which cause inflammation and fibrosis

Secondary retroperitoneal fibrosis

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

Pathophysiology

Clinical features

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

Diagnostics

References:[3][1]

Treatment

Medical therapy[4][11]

  • 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

Prognosis

  • Prognosis of non-malignancy induced RPF is good, with symptomatic and clinical improvement obvious within a few weeks of initiating therapy. [1][11]
  • High recurrence rates of idiopathic RPF (70%)[11]
  • Poor prognosis of malignancy-incduced RPF (∼ 6 months)[13]
  • 1. Biyani CS. Retroperitoneal Fibrosis. In: Schwartz BF. Retroperitoneal Fibrosis. New York, NY: WebMD. http://emedicine.medscape.com/article/458501. Updated July 31, 2016. Accessed January 16, 2017.
  • 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): pp. 423–431. doi: 10.2214/AJR.07.3629.
  • 3. Vaglio A, Palmisano A. Clinical manifestations and diagnosis of retroperitoneal fibrosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-retroperitoneal-fibrosis. Last updated September 4, 2015. Accessed January 16, 2017.
  • 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): pp. 15–21. doi: 10.1016/j.revmed.2014.10.008.
  • 5. Mann DA, Oakley F. Serotonin paracrine signaling in tissue fibrosis. Biochim Biophys Acta. 2013; 1832(7): pp. 905–910. doi: 10.1016/j.bbadis.2012.09.009.
  • 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): pp. 535–552. doi: 10.1148/rg.332125085.
  • 7. Tzou M, Gazeley DJ, Mason PJ. Retroperitoneal fibrosis. Vasc Med. 2014; 19(5): pp. 407–414. doi: 10.1177/1358863X14546160.
  • 8. 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): pp. 338–346. doi: 10.1016/S0140-6736(11)60934-3.
  • 9. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet. 2006; 367(9506): pp. 241–251. doi: 10.1016/S0140-6736(06)68035-5.
  • 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): pp. 454–462. doi: 10.1016/S0002-9343(03)00056-1.
  • 11. Vaglio A, Palmisano A. Treatment of retroperitoneal fibrosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/treatment-of-retroperitoneal-fibrosis. Last updated January 14, 2016. Accessed January 16, 2017.
  • 12. Tiptaft RC, Costello AJ, Paris AM, Blandy JP. The long-term follow-up of idiopathic retroperitoneal fibrosis. Br J Urol. 1982; 54(6): pp. 620–624. pmid: 7150912.
  • 13. Arrivé L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology. 1989; 172(1): pp. 139–143. doi: 10.1148/radiology.172.1.2740497.
  • Herold G. Internal Medicine. Cologne, Germany: Herold G; 2014.
last updated 02/20/2018
{{uncollapseSections(['qhaCU4', '7ha424', 'HhaK24', 'GhaB24', 'thaXf4', 'uhapf4', 'Dha1T4', 'w3chkX0'])}}