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]
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]
Treatmentedit
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 modulatortamoxifen has shown to improve the condition in various small trials, although the exact mechanism of its action remains unclear.[25]
Referencesedit
^ abcvan Bommel EF (July 2002). "Retroperitoneal fibrosis". Neth J Med. 60 (6): 231–42. PMID 12365466. Retrieved 10 August 2022.
^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. PMC4049060. PMID 24926452.
^ abcVaglio 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.
^ abLian, 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. PMC7322295. PMID 32637930.
^ abBiyani, 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.
^ abcdVaglio, 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. PMC4926988. 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 & Rheumatism (Arthritis Care & 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. PMC3068889. PMID 21454732.
^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. PMC4087819. 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 Sjögren's syndrome: A case report". Medicine. 97 (26): e11303. doi:10.1097/MD.0000000000011303. PMC6039603. PMID 29953016. e11303.
^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.
^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 & Management". Medscape. Retrieved 10 August 2022.