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]

Retroperitoneal fibrosis
SpecialtyUrology 

Causes

Its association with various immune-related conditions and response to immunosuppression have led to speculation regarding an autoimmune cause 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 the results of laboratory studies. CT is the best diagnostic modality:[6] a confluent mass surrounding the aorta[5] and common iliac arteries can be seen. On MRI, it has low T1 signal intensity and variable T2 signal. Malignant retroperitoneal fibrosis usually give uneven MRI signals, bulky, extend above the origins of renal arteries, or displace aorta anteriorly. Besides, malignant retroperitoneal fibrosis less frequently displaces ureter medially when compared to other retroperitoneal fibrosis.[7]

On fludeoxyglucose (18F) (FDG) positron emission tomography (PET) scan, it will show accumulation of FDG in the affected area.[7]

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

References
  1. Albarran-Ormond syndrome at Who Named It?
  2. Ormond JK (1948). "Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process". J. Urol. 59 (6): 1072–9. doi:10.1016/s0022-5347(17)69482-5. PMID 18858051.
  3. Ormond JK (October 1965). "Idiopathic retroperitoneal fibrosis: a discussion of the etiology". J. Urol. 94 (4): 385–90. doi:10.1016/s0022-5347(17)63635-8. PMID 5839568.
  4. Thongprayoon, C; Spanuchart I; Cheungpasitporn W; Kangwanpornsiri A (May 2014). "Idiopathic retroperitoneal fibrosis: a challenging case in a rare disease". N Am J Med Sci. 6 (5): 237–8. doi:10.4103/1947-2714.132945. PMC 4049060. PMID 24926452.
  5. Vaglio A, Salvarani C, Buzio C (January 2006). "Retroperitoneal fibrosis". Lancet. 367 (9506): 241–51. doi:10.1016/S0140-6736(06)68035-5. PMID 16427494.
  6. Avaria P, Hirsch M.Forniceal rupture and urinoma secondary to retroperitoneal fibrosis: A clinical case and literature review. https://doi.org/10.1016/j.uromx.2015.10.001
  7. Vaglio, Augusto; Maritati, Federica (July 2016). "Idiopathic Retroperitoneal Fibrosis". Journal of the American Society of Nephrology. 27 (7): 1880–1889. doi:10.1681/ASN.2015101110. ISSN 1046-6673. PMC 4926988. PMID 26860343.
  8. van Bommel EF (July 2002). "Retroperitoneal fibrosis". Neth J Med. 60 (6): 231–42. PMID 12365466.
Classification
External resources
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