Retroperitoneal fibrosis

Retroperitoneal fibrosis
Classification and external resources
Specialty	urology
ICD-10	N13.5
ICD-9-CM	593.4
DiseasesDB	11445
MedlinePlus	000463
eMedicine	radio/605 med/3664
MeSH	D012185

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

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. PMID 18858051.
3. Ormond JK (October 1965). "Idiopathic retroperitoneal fibrosis: a discussion of the etiology". J Urol. 94 (4): 385–90. 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: 237–8. doi:10.4103/1947-2714.132945. 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. van Bommel EF (July 2002). "Retroperitoneal fibrosis". Neth J Med 60 (6): 231–42. PMID 12365466.