Retroperitoneal fibrosis

Last updated
Retroperitoneal fibrosis
Other namesOrmond'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 (fibrosis) 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]

Contents

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] However, emerging evidence suggests that occupational exposure to asbestos and tobacco smoking are also strong, synergistic risk factors for the development of idiopathic RPF, underscoring the importance of preventative measures in high risk populations. [10]

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: [25] 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. [14]

On fludeoxyglucose (18F) (FDG) positron emission tomography (PET) scan, FDG accumulation is shown in the affected area. [14]

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] Glucocorticoids act as anti-inflammatory agents, diminishing the size of the retroperitoneal mass and alleviating obstructions and associated complications; this is achieved through the suppression of cytokine synthesis that contributes to the acute-phase reaction, and by impeding the development of collagen. [26] 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. [27]

Related Research Articles

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<span class="mw-page-title-main">Autoimmune hepatitis</span> Chronic, autoimmune disease of the liver

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<span class="mw-page-title-main">Biliary atresia</span> Absence, blockage, or narrowing of bile ducts in the liver at birth

Biliary atresia, also known as extrahepatic ductopenia and progressive obliterative cholangiopathy, is a childhood disease of the liver in which one or more bile ducts are abnormally narrow, blocked, or absent. It can be congenital or acquired. It has an incidence of one in 10,000–15,000 live births in the United States, and a prevalence of one in 16,700 in the British Isles. Biliary atresia is most common in East Asia, with a frequency of one in 5,000.

<span class="mw-page-title-main">Primary biliary cholangitis</span> Autoimmune disease of the liver

Primary biliary cholangitis (PBC), previously known as primary biliary cirrhosis, is an autoimmune disease of the liver. It results from a slow, progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up in the liver, a condition called cholestasis. Further slow damage to the liver tissue can lead to scarring, fibrosis, and eventually cirrhosis.

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<span class="mw-page-title-main">Acute pancreatitis</span> Medical condition

Acute pancreatitis (AP) is a sudden inflammation of the pancreas. Causes include a gallstone impacted in the common bile duct or the pancreatic duct, heavy alcohol use, systemic disease, trauma, elevated calcium levels, hypertriglyceridemia, certain medications, hereditary causes and, in children, mumps. Acute pancreatitis may be a single event, it may be recurrent, or it may progress to chronic pancreatitis and/or pancreatic failure.

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<span class="mw-page-title-main">Idiopathic sclerosing mesenteritis</span> Medical condition

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<span class="mw-page-title-main">IgG4-related disease</span> Medical condition

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<span class="mw-page-title-main">IgG4-related ophthalmic disease</span> Medical condition

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References

  1. 1 2 3 van Bommel EF (July 2002). "Retroperitoneal fibrosis". Neth J Med. 60 (6): 231–42. PMID   12365466 . Retrieved 10 August 2022.
  2. ↑ Albarran-Ormond syndrome at Who Named It?
  3. ↑ 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.
  4. ↑ 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.
  5. ↑ 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.
  6. 1 2 3 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. S2CID   25511135.
  7. ↑ Kamisawa, Terumi; Egawa, Naoto; Nakajima, Hitoshi (2003). "Autoimmune Pancreatitis is a Systemic Autoimmune Disease". American Journal of Gastroenterology. 98 (12): 2811–2812. doi:10.1111/j.1572-0241.2003.08758.x. PMID   14687846. S2CID   36222260.
  8. ↑ Lanzillotta, Marco; Mancuso, Gaia; Della-Torre, Emmanuel (2020). "Advances in the diagnosis and management of IgG4 related disease". The British Medical Journal. 369: m1067. doi: 10.1136/bmj.m1067 . PMID   32546500. m1067.
  9. 1 2 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. doi: 10.1111/1756-185X.12863 . PMID   27125330.
  10. ↑ Goldoni, Matteo; Bonini, Silvia; Urban, Maria L.; Palmisano, Alessandra; De Palma, Giuseppe; Galletti, Elisa; Coggiola, Marco; Buzio, Carlo; Mutti, Antonio; Vaglio, Augusto (2014-08-05). "Asbestos and Smoking as Risk Factors for Idiopathic Retroperitoneal Fibrosis: A Case–Control Study". Annals of Internal Medicine. 161 (3): 181. doi:10.7326/M13-2648. ISSN   0003-4819.
  11. ↑ Dhanawat, Aditya; Gupta, Partisha; Padhan, Prasanta (2020). "Mixed Connective Tissue Disease with Retroperitoneal Fibrosis: A Rare Occurrence". Archives of Rheumatology. 35 (1): 137–141. doi: 10.5606/ArchRheumatol.2020.7452 . PMC   7322295 . PMID   32637930.
  12. 1 2 Biyani, Chandra Shekhar; Taylor, Joby; Browning, Anthony J. (2 September 2021). Schwartz, Bradley Fields (ed.). "Retroperitoneal Fibrosis Overview". Medscape. Retrieved 10 August 2022.
  13. ↑ 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. doi:10.1097/00003072-200206000-00005. PMID   12045432. S2CID   423062.
  14. 1 2 3 4 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.
  15. ↑ LeBlanc, Claire M.A.; Inman, Robert D.; Dent, Peter; Smith, Charles; Babyn, Paul; Laxer, Ronald M. (2002). "Retroperitoneal Fibrosis: An Extraarticular Manifestation of Ankylosing Spondylitis". Arthritis & Rheumatism. 42 (2): 210–214. doi: 10.1002/art.10267 . PMID   11954016.
  16. ↑ 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. doi: 10.4065/mcp.2010.0663 . PMC   3068889 . PMID   21454732.
  17. ↑ 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. doi: 10.1093/omcr/omy056 . PMC   6109199 . PMID   30159151.
  18. ↑ 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. doi:10.2214/ajr.181.4.1810993. PMID   14500215 . Retrieved 10 August 2022.
  19. ↑ 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. doi: 10.3748/wjg.v12.i18.2955 . PMC   4087819 . PMID   16718827.
  20. ↑ Tatari, Murat; Trujillo, David Abia; Mira-Avendano, Isabel (October 2020). "Retroperitoneal Fibrosis Presenting as Ureteral Obstruction Secondary to Sarcoidosis". Chest. 158 (4): A1200. doi: 10.1016/j.chest.2020.08.1093 . S2CID   225126556. A1200.
  21. ↑ 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. doi: 10.1097/MD.0000000000011303 . PMC   6039603 . PMID   29953016. e11303.
  22. ↑ 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. doi:10.1001/archinte.1985.00360110200042. PMID   4062468.
  23. ↑ Poredos, Pavel (2008). "Inflammatory aortic aneurysm". e-Journal of the European Society for Cardiology Practice. 7 (10). Retrieved 10 August 2022.
  24. ↑ 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. doi: 10.1161/CIRCULATIONAHA.114.010173 . PMID   25287771.
  25. ↑ 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
  26. ↑ Vaglio, Augusto; Salvarani, Carlo; Buzio, Carlo (January 2006). "Retroperitoneal fibrosis". The Lancet. 367 (9506): 241–251. doi:10.1016/s0140-6736(06)68035-5. ISSN   0140-6736.
  27. ↑ Biyani, Chandra Shekhar; Taylor, Joby; Browning, Anthony J. (2 September 2021). Schwartz, Bradley Fields (ed.). "Retroperitoneal Fibrosis Treatment & Management". Medscape. Retrieved 10 August 2022.
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