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Retroperitoneal fibrosis

January 01, 1970

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]

Retroperitoneal fibrosis
Other namesOrmond's disease
SpecialtyRheumatology, vascular surgery, urology
Risk factorsmale sex, age[1]

Causes edit

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 edit

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 edit

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 edit

  1. ^ a b c 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. ^ a b c 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. ^ 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. doi:10.1111/1756-185X.12863. PMID 27125330.
  10. ^ 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.
  11. ^ 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.
  12. ^ 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.
  13. ^ a b c d 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.
  14. ^ 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 (Arthritis Care & Research). 42 (2): 210–214. doi:10.1002/art.10267. PMID 11954016.
  15. ^ 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.
  16. ^ 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.
  17. ^ 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.
  18. ^ 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.
  19. ^ 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.
  20. ^ 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.
  21. ^ 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.
  22. ^ Poredos, Pavel (2008). "Inflammatory aortic aneurysm". e-Journal of the European Society for Cardiology Practice. 7 (10). Retrieved 10 August 2022.
  23. ^ 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.
  24. ^ 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
  25. ^ Biyani, Chandra Shekhar; Taylor, Joby; Browning, Anthony J. (2 September 2021). Schwartz, Bradley Fields (ed.). "Retroperitoneal Fibrosis Treatment & Management". Medscape. Retrieved 10 August 2022.

External links edit

January 01, 1970
retroperitoneal, fibrosis, ormond, disease, disease, featuring, proliferation, fibrous, tissue, retroperitoneum, compartment, body, containing, kidneys, aorta, renal, tract, various, other, structures, present, with, lower, back, pain, kidney, failure, hyperte. 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 Retroperitoneal fibrosisOther namesOrmond s diseaseSpecialtyRheumatology vascular surgery urologyRisk factorsmale sex age 1 Contents 1 Causes 2 Diagnosis 3 Treatment 4 References 5 External linksCauses editThe 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 connective tissue disease 10 11 Riedel s thyroiditis 12 sclerosing cholangitis citation needed membranous nephropathy 13 ankylosing spondylitis 14 inflammatory bowel disease 11 15 ANCA associated vasculitis 13 autoimmune pancreatitis 16 17 18 sarcoidosis 19 primary biliary cirrhosis 20 21 inflammatory abdominal aortic aneurysm 22 23 Diagnosis editThe 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 editIn 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 edit a b c van Bommel EF July 2002 Retroperitoneal fibrosis Neth J Med 60 6 231 42 PMID 12365466 Retrieved 10 August 2022 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 doi 10 1016 s0022 5347 17 69482 5 PMID 18858051 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 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 a b c 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 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 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 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 doi 10 1111 1756 185X 12863 PMID 27125330 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 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 doi 10 1097 00003072 200206000 00005 PMID 12045432 S2CID 423062 a b c d 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 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 amp Rheumatism Arthritis Care amp Research 42 2 210 214 doi 10 1002 art 10267 PMID 11954016 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 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 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 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 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 Huang Xuan Lu Bin Li Meng Fan Yihong Zhang Lu 2018 IgG4 related retroperitoneal fibrosis overlapping with primary biliary cirrhosis and primary Sjogren s syndrome A case report Medicine 97 26 e11303 doi 10 1097 MD 0000000000011303 PMC 6039603 PMID 29953016 e11303 Sevenet Francois 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 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 doi 10 1161 CIRCULATIONAHA 114 010173 PMID 25287771 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 Retroperitoneal Fibrosis Treatment amp Management Medscape Retrieved 10 August 2022 External links edit Retrieved from https en wikipedia org w index php title Retroperitoneal fibrosis amp oldid 1174279884, wikipedia, wiki, book, books, library,

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