Retroperitoneal Fibrosis; a Single-centre Case Experience with Literature Review

Overview

Journal Rheumatol Adv Pract

Publisher Oxford University Press

Specialty Rheumatology

Date 2019 Aug 22

PMID 31431986

Citations 5

Authors

Saqib Adnan

Aicha Bouraoui

Sampi Mehta

Siwalik Banerjee

Shaifali Jain

Bhaskar Dasgupta

Affiliations

Soon will be listed here.

Abstract

Objective: We present 13 patients with retroperitoneal fibrosis, focusing on clinical features, radiological characteristics, treatments and their outcomes.

Methods: Retrospective review of the medical records was performed of all retroperitoneal fibrosis patients diagnosed and treated in our department between 2012 and 2017.

Results: Twelve patients were male, with a median age of 64 years. Eleven patients presented with abdominal pain or back pain or both. Aetiologies varied from idiopathic to malignancy and vasculitis. Twelve patients had PET scans. These showed F-fluorodeoxyglucose-avid retroperitoneal soft tissue around the abdominal aorta in the vast majority, with five scans also demonstrating localized or generalized uptake by the aorta. In all cases except one, glucocorticoids were applied as the first-line therapy. Further immunosuppressive therapy was required in 10 cases.

Conclusion: Our patients were male and older in age compared with the existing literature. PET scans were very helpful in diagnosis of retroperitoneal fibrosis. Rituximab was found to be an effective treatment in six of our patients.

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References

Drieskens O, Blockmans D, Van den Bruel A, Mortelmans L . Riedel's thyroiditis and retroperitoneal fibrosis in multifocal fibrosclerosis: positron emission tomographic findings. Clin Nucl Med. 2002; 27(6):413-5. DOI: 10.1097/00003072-200206000-00005. View

Kardar A, Kattan S, Lindstedt E, Hanash K . Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol. 2002; 168(2):550-5. View

Marcolongo R, Tavolini I, Laveder F, Busa M, Noventa F, Bassi P . Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med. 2004; 116(3):194-7. DOI: 10.1016/j.amjmed.2003.08.033. View

Uibu T, Oksa P, Auvinen A, Honkanen E, Metsarinne K, Saha H . Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet. 2004; 363(9419):1422-6. DOI: 10.1016/S0140-6736(04)16100-X. View

Vaglio A, Versari A, Fraternali A, Ferrozzi F, Salvarani C, Buzio C . (18)F-fluorodeoxyglucose positron emission tomography in the diagnosis and followup of idiopathic retroperitoneal fibrosis. Arthritis Rheum. 2005; 53(1):122-5. DOI: 10.1002/art.20914. View

Salvarani C, Pipitone N, Versari A, Vaglio A, Serafini D, Bajocchi G . Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum. 2005; 53(2):298-303. DOI: 10.1002/art.21074. View

Vaglio A, Salvarani C, Buzio C . Retroperitoneal fibrosis. Lancet. 2006; 367(9506):241-51. DOI: 10.1016/S0140-6736(06)68035-5. View

van Bommel E, Siemes C, Hak L, van der Veer S, Hendriksz T . Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis. 2007; 49(5):615-25. DOI: 10.1053/j.ajkd.2007.02.268. View

Corradi D, Maestri R, Palmisano A, Bosio S, Greco P, Manenti L . Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int. 2007; 72(6):742-53. DOI: 10.1038/sj.ki.5002427. View

10.

Fry A, Singh S, Gunda S, Boustead G, Hanbury D, McNicholas T . Successful use of steroids and ureteric stents in 24 patients with idiopathic retroperitoneal fibrosis: a retrospective study. Nephron Clin Pract. 2008; 108(3):c213-20. DOI: 10.1159/000119715. View

11.

ORMOND J . Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol. 1948; 59(6):1072-9. DOI: 10.1016/S0022-5347(17)69482-5. View

12.

van Bommel E, Jansen I, Hendriksz T, Aarnoudse A . Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore). 2009; 88(4):193-201. DOI: 10.1097/MD.0b013e3181afc420. View

13.

Brandt A, Kamper L, Kukuk S, Haage P, Roth S . Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry. J Urol. 2010; 185(2):526-31. DOI: 10.1016/j.juro.2010.09.105. View

14.

Kermani T, Crowson C, Achenbach S, Luthra H . Idiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc. 2011; 86(4):297-303. PMC: 3068889. DOI: 10.4065/mcp.2010.0663. View

15.

Khosroshahi A, Carruthers M, Stone J, Shinagare S, Sainani N, Hasserjian R . Rethinking Ormond's disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore). 2013; 92(2):82-91. PMC: 4553983. DOI: 10.1097/MD.0b013e318289610f. View

16.

Oliveira Caiafa R, Sierra Vinuesa A, Salvador Izquierdo R, Brufau B, Ayuso Colella J, Nicolau Molina C . Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics. 2013; 33(2):535-52. DOI: 10.1148/rg.332125085. View

17.

Alberici F, Palmisano A, Urban M, Maritati F, Oliva E, Manenti L . Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis. Ann Rheum Dis. 2013; 72(9):1584-6. DOI: 10.1136/annrheumdis-2013-203267. View

18.

Ezimora A, Faulkner M, Adebiyi O, Ogungbemile A, Marianna S, Nzerue C . Retroperitoneal fibrosis: a rare cause of acute renal failure. Case Rep Nephrol. 2014; 2012:645407. PMC: 3914163. DOI: 10.1155/2012/645407. View

19.

Bakir B, Yilmaz F, Turkay R, Ozel S, Bilgic B, Velioglu A . Role of diffusion-weighted MR imaging in the differentiation of benign retroperitoneal fibrosis from malignant neoplasm: preliminary study. Radiology. 2014; 272(2):438-45. DOI: 10.1148/radiol.14131565. View

20.

Gaertner S, Cordeanu E, Mirea C, Stephan D . [Retroperitoneal fibrosis]. Presse Med. 2014; 43(9):1021-3. DOI: 10.1016/j.lpm.2014.02.019. View