Case Reports

Idiopathic retroperitoneal fibrosis: A case report and review of articles

Abstract

Idiopathic retroperitoneal fibrosis is a rare disorder of an unknown etiology characterized by an inflammatory proliferative fibrosing process that may involve the ureters in 80-100% of cases.  The present study was carried on a 38 years old man who was admitted to Imam Khomeini Hospital with severe abdominal pain and renal failure. Abdominal MRI showed encasement of abdominal aorta and bilateral hydroureteronephrosis. Tissue biopsy established the diagnosis of retroperitoneal fibrosis and ureteral obstruction was managed by insertion of bilateral ureteral stents. Presupposing the un-resectability, medical therapy was started. However he didn’t show objective response to Prednisolone (1mg/kg) and had adverse effects. Subsequently, his disease was controlled by adding mycophenolate mofetil and azathioprine to reduce the steroid dose. After a few months, urinary stents were removed and he had been on complete remission for more than 4 years. Although advanced idiopathic retroperitoneal fibrosis would be effectively treated by a combination of ureteric stents and steroids, in difficult cases, second-line treatment with other immunosuppressive drugs may help to achieve long-term remission of disease.

Vaglio A, Buzio C. Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol. 2005 Jan;17(1):34-40.

Pipitone N, Salvarani C, Peter HH. [Chronic periaortitis]. Internist (Berl). 2010 Jan;51(1):45-52.

Vogt B, Meier P, Burnier M. [Retroperitoneal fibrosis, M. Ormond, periaoartitis, ...?]. Ther Umsch. 2008 May;65(5):265-8.

Parums DV, Brown DL, Mitchinson MJ. Serum antibodies to oxi- dized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med. 1990 Apr;114(4):383-7.

Wolyniec W, Kozuchowska M, Sworczak K, Jezior D, Rozanska- Kluziak A, Rudka R, et al. When can we really diagnose idiopathic retroperitoneal fibrosis? Nephrol Dial Transplant. 2007 Jun;22(6):1786; author reply -7.

Vaglio A, Greco P, Corradi D, Palmisano A, Martorana D, Ronda N, et al. Autoimmune aspects of chronic periaortitis. Autoimmun Rev. 2006 Aug;5(7):458-64.

Stifelman MD, Shah O, Mufarrij P, Lipkin M. Minimally invasive management of retroperitoneal fibrosis. Urology. 2008 Feb;71(2):201-4.

Moroni G, Gallelli B, Banfi G, Sandri S, Messa P, Ponticelli C. Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches. Nephrol Dial Transplant. 2006 Sep;21(9):2485-90.

Ormond JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol. 1948 Jun;59(6):1072-9.

Disel U, Alpay NR, Paydas S. Retroperitoneal fibrosis secondary to different etiologies (hemilaminectomy and hypothyroidism): reports of two cases. Ren Fail. 2007;29(5):639-46.

Lee JE, Han SH, Kim DK, Moon SJ, Choi KH, Lee HY, et al. Idio- pathic retroperitoneal fibrosis associated with Hashimoto’s thyroiditis in an old-aged man. Yonsei Med J. 2008 Dec 31;49(6):1032-5.

Surabhi VR, Menias C, Prasad SR, Patel AH, Nagar A, Dalrymple NC. Neoplastic and non-neoplastic proliferative disorders of the perire- nal space: cross-sectional imaging findings. Radiographics. 2008 Jul- Aug;28(4):1005-17.

Rodriguez Jornet A, Andreu Navarro FJ, Orellana Fernandez R, Ibeas Lopez J, Garcia Garcia M. [Idiopathic retroperitoneal fibrosis: clinico-pathological characteristics]. Nefrologia. 2009;29(4):298-303.

Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002 Apr 20;359(9315):1403-4.

Yamamoto M, Takahashi H, Naishiro Y, Isshiki H, Ohara M, Suzuki C, et al. Mikulicz’s disease and systemic IgG4-related plasmacytic syn- drome (SIPS). Nihon Rinsho Meneki Gakkai Kaishi. 2008 Feb;31(1):1- 8.

Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as mani- festations of IgG4-related systemic disease. Curr Opin Rheumatol. 2011 Jan;23(1):88-94.

Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med. 2006;4:23.

Miura H, Miyachi Y. IgG4-related retroperitoneal fibrosis and sclerosing cholangitis independent of autoimmune pancreatitis. A recurrent case after a 5-year history of spontaneous remission. JOP. 2009;10(4):432-7.

Duchene DA, Winfield HN, Cadeddu JA, Clayman RV, Gomella LG, Kavoussi LR, et al. Multi-institutional survey of laparoscopic ure- terolysis for retroperitoneal fibrosis. Urology. 2007 Jun;69(6):1017-21.

Fry AC, Singh S, Gunda SS, Boustead GB, Hanbury DC, McNicho- las TA, et al. 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.

Adler S, Lodermeyer S, Gaa J, Heemann U. Successful mycophe- nolate mofetil therapy in nine patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford). 2008 Oct;47(10):1535-8.

Swartz RD. Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment. Am J Kidney Dis. 2009 Sep;54(3):546-53.

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IssueVol 3 No 2 (2011) QRcode
SectionCase Reports
Keywords
retroperitoneal fibrosis ureteral obstruction medical therapy
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How to Cite
1.
Sadighi S, Ayati M, Mohagheghi MA, Shahriaran S, Jahanzad I, Safavi S. Idiopathic retroperitoneal fibrosis: A case report and review of articles. Basic Clin Cancer Res. 2016;3(2):20-25.