Medical condition
Retroperitoneal fibrosis Other names Ormond's disease
Specialty Rheumatology, vascular surgery, urology
Risk factors male sex, age
[1]
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.
[2]
[3]
[4]
Causes
The association of idiopathic retroperitoneal fibrosis with various immune-related conditions and response to
immunosuppression led to a search for an
autoimmune cause of idiopathic RPF.
[5]
[6] Many of these previously idiopathic cases can now be attributed to
IgG4-related disease , an autoimmune disorder proposed in 2003.
[7]
[8]
[9] Otherwise, one-third of cases are secondary to
malignancy , medication (
methysergide ,
hydralazine ,
beta blockers ), prior
radiotherapy , or certain infections.
[1]
[9]
Other associations include:
Diagnosis
The diagnosis of retroperitoneal fibrosis cannot be made on the basis of the results of laboratory studies. CT is the best diagnostic modality:
[24] a confluent mass surrounding the aorta
[6] and common iliac arteries can be seen. On MRI, it has low T1 signal intensity and variable T2 signal. Malignant retroperitoneal fibrosis usually gives uneven MRI signals, is bulky, extends above the origins of renal arteries, or displaces the aorta anteriorly. Additionally, malignant retroperitoneal fibrosis less frequently displaces the ureters medially when compared to other causes of retroperitoneal fibrosis.
[13]
On
fludeoxyglucose (18F) (FDG)
positron emission tomography (PET) scan, FDG accumulation is shown in the affected area.
[13]
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.
[6]
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.
[1] The
selective estrogen receptor modulator
tamoxifen has shown to improve the condition in various small trials, although the exact mechanism of its action remains unclear.
[25]
References
^
a
b
c van Bommel EF (July 2002).
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^
Albarran-Ormond syndrome at
Who Named It?
^ Ormond JK (1948). "Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process". J. Urol . 59 (6): 1072–9.
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^ Ormond JK (October 1965). "Idiopathic retroperitoneal fibrosis: a discussion of the etiology". J. Urol . 94 (4): 385–90.
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^ 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.
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^
a
b
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^ Kamisawa, Terumi; Egawa, Naoto; Nakajima, Hitoshi (2003). "Autoimmune Pancreatitis is a Systemic Autoimmune Disease". American Journal of Gastroenterology . 98 (12): 2811–2812.
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^ Lanzillotta, Marco; Mancuso, Gaia; Della-Torre, Emmanuel (2020).
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^
a
b Lian, Linjuan; Wang, Cong; Tian, Jian-li (2016).
"IgG4-related retroperitoneal fibrosis: A newly characterized disease" . International Journal of Rheumatic Diseases . 19 (11): 1049–1055.
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^ Dhanawat, Aditya; Gupta, Partisha; Padhan, Prasanta (2020).
"Mixed Connective Tissue Disease with Retroperitoneal Fibrosis: A Rare Occurrence" . Archives of Rheumatology . 35 (1): 137–141.
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^
a
b Biyani, Chandra Shekhar; Taylor, Joby; Browning, Anthony J. (2 September 2021). Schwartz, Bradley Fields (ed.).
"Retroperitoneal Fibrosis Overview" . Medscape . Retrieved 10 August 2022 .
^ Drieskens, Oliver; Blockmans, Daniel; Van den Bruel, Annick; Mortelmans, Luc (2002). "Riedel's thyroiditis and retroperitoneal fibrosis in multifocal fibrosclerosis: Positron emission tomographic findings". Clinical Nuclear Medicine . 27 (6): 413–415.
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^
a
b
c
d Vaglio, Augusto; Maritati, Federica (July 2016).
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doi :
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ISSN
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PMC
4926988 .
PMID
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^ LeBlanc, Claire M.A.; Inman, Robert D.; Dent, Peter; Smith, Charles; Babyn, Paul; Laxer, Ronald M. (2002).
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^ Kermani, Tanaz A.; Crowson, Cynthia S.; Achenbach, Sara J.; Luthra, Harvinder S. (2011).
"Idiopathic Retroperitoneal Fibrosis: A Retrospective Review of Clinical Presentation, Treatment, and Outcomes" . Mayo Clinic Proceedings . 86 (4): 297–303.
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^ Fanidou, Domna; Filippou, Nikolaos; Katseli, Anastasia; Papadopoulos, Georgios; Skandalakis, Panagiotis; Filippou, Dimitrios (2018).
"Autoimmune pancreatitis associated with retroperitoneal fibrosis mimicking cholangiocarcinoma" . Oxford Medical Case Reports . 2018 (9): omy056.
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^ Fukukura, Yoshihiko; Fujiyoshi, Fumito; Nakamura, Fumihiko; Hamada, Hiroyuki; Nakajo, Masayuki (2003).
"Autoimmune Pancreatitis Associated with Idiopathic Retroperitoneal Fibrosis" . American Journal of Roentgenology (PDF). 181 (4): 993–995.
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^ Kamisawa, Terumi; Chen, Pong-Yui; Tu, Yuyang; Nakajima, Hitoshi; Egawa, Naoto (2006).
"Autoimmune pancreatitis metachronously associated with retroperitoneal fibrosis with IgG4-positive plasma cell infiltration" . World Journal of Gastroenterology . 12 (18): 2955–2957.
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^ Tatari, Murat; Trujillo, David Abia; Mira-Avendano, Isabel (October 2020).
"Retroperitoneal Fibrosis Presenting as Ureteral Obstruction Secondary to Sarcoidosis" . Chest . 158 (4): A1200.
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225126556 . A1200.
^ Huang, Xuan; Lu, Bin; Li, Meng; Fan, Yihong; Zhang, Lu (2018).
"IgG4-related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjögren's syndrome: A case report" . Medicine . 97 (26): e11303.
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^ Sevenet, François; Capron-Chivrac, Dominique; Delcenserie, Richard; Lelarge, Christian; Delamarre, Jacques; Capron, Jean-Pierre (1985). "Idiopathic Retroperitoneal Fibrosis and Primary Biliary Cirrhosis: A New Association?". Archives of Internal Medicine . 145 (11): 2124–2125.
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^ Poredos, Pavel (2008).
"Inflammatory aortic aneurysm" . e-Journal of the European Society for Cardiology Practice . 7 (10). Retrieved 10 August 2022 .
^ Fatima, Javairiah; Gota, Carmen; Clair, Daniel G.; Billings, Steven D.; Gornik, Heather L. (2014).
"Inflammatory Abdominal Aortic Aneurysm with Retroperitoneal Fibrosis" . Circulation . 130 (15): 1300–1302.
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^ 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
^ Biyani, Chandra Shekhar; Taylor, Joby; Browning, Anthony J. (2 September 2021). Schwartz, Bradley Fields (ed.).
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External links
Classification External resources