Surviving Complications of Retroperitoneal Fibrosis with Intermittent Relapses over a Period of 20 years: Case report and literature Review Is it chronic progressive disease or intermittent relapses every time encasing a new structure?

Surviving Complications of Retroperitoneal Fibrosis with Intermittent Relapses over a Period of 20 years: Case report and literature Review
Is it chronic progressive disease or intermittent relapses every time encasing a new structure?

Main Article Content

Dr. Sunita Dinkar
Dr. Srajan Dashore
Dr. Sunil Joshi
Dr. Himanshu Shah
Dr. Tushar Shah


Idiopathic retroperitoneal fibrosis is a rare fibro-inflammatory disease of varied etiology which usually originates around aorta and spreads caudally along Iliac vessels into adjacent
retroperitoneum causing ureteral obstruction as the most frequent complication.
A 53-year-old male patient presented with complaint of mild pain in both the legs off and on. On investigating further, we found that he had been struggling with intermittent relapses every 3-4 years for last 20 years since he was first diagnosed with Idiopathic Retroperitoneal Fibrosis. He was
33-year-old when he first developed the symptoms of anuria for 48 hours and was diagnosed with Idiopathic retroperitoneal fibrosis. This was followed by atrophy of left kidney and hypertension 6 years later, then hypothyroidism after another 3years and finally involvement of Inferior Vena
Cava and acute Deep Vein Thrombosis of lower limbs after another 3-4 years. His deep vein thrombosis was well managed in time. He was put on glucocorticoids everytime he had a relapse and a complication.
We did a review of literature to understand recent advances about its pathogenesis, diagnosis, investigations and management. We searched in PubMed using terms like retroperitoneal fibrosis
alone and in combination with related terms such as Inferior Vena Cava thrombosis, Deep Vein Thrombosis, Tamoxifen, Methotrexate. This case is unique as it is very rare to find acute Deep Vein Thrombosis in Idiopathic retroperitoneal fibrosis without development of any collaterals
when Inferior Vena Cava lumen is compromised to almost complete obstruction. After a follow up of 20 years patient is doing well in terms of physical activity and psychological
wellbeing with anti-hypertensives, thyroxine and anti-coagulants. Is the disease-free interval actually free of the disease or it just subsided with immunosuppressants to become active after some time?


Boiardi L, Vaglio A, Nicoli D, Farnetti E, Palmisano A, Pipitone N, Maritati F, Casali B, Martorana D, Moroni G, Gallelli B, Buzio C, Salvarani C. (2011) CC chemokine receptor 5 polymorphism in chronic periaortitis. Rheumatology (Oxford). Jun;50(6):1025-32. doi: 10.1093/rheumatology/keq416. Epub 2011 Jan 21. PMID: 21258050.

Brandt AS, Kamper L, Kukuk S, Haage P, Roth S. (2014) Tamoxifen monotherapy in the treatment of retroperitoneal fibrosis. Urol Int. Jul ;2014;93(3):320-5. doi: 10.1159/000357814. Epub 26. PMID: 25073896.

Caiafa RO , Vinuesa AS, Izquierdo RS, Brufau BP, Colella JRA, Molina CN. (2013) Retroperitoneal fibrosis: role of imaging in diagnosis and follow-up. Radiographics; Mar-Apr 33(2):535-52.

Choi SH, Lim HK, Lee WJ. (1997) Idiopathic Retroperitoneal Fibrosis with Rectosigmoid obstruction : Imaging Findings ; J Korean Radiol Soc 1997;37:881-883.

Corradi D, Maestri R, Palmisano A, Bosio S, Greco P, Manenti L, Ferretti S, Cobelli R, Moroni G, Dei Tos AP, Buzio C, Vaglio A. (2007) Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int. 2007 Sep;72(6):742-53. doi: 10.1038/ Epub PMID: 17622270.

Couderc M, Mathieu S, Dubost JJ, Soubrier M. (2013) Retroperitoneal fibrosis during etanercept therapy for rheumatoid arthritis. J Rheumatol. Nov;40(11):1931-3. doi: 10.3899/jrheum.130324. PMID: 24187163.

Cronin CG, Lohan DG, Blake MA, Roche C, McCarthy P, Murphy JM. (2008) Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol. Aug;191(2):423-31.

Diamond EL, Dagna L, Hyman DM, Cavalli G, Janku F, Estrada-Veras J, Ferrarini M, Abdel-Wahab O, Heaney ML, Scheel PJ, Feeley NK, Ferrero E, McClain KL, Vaglio A, Colby T, Arnaud L, Haroche J. (2014) Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease. Blood. Jul ;24;124(4):483-92. doi: 10.1182/blood-2014-03-561381. Epub 2014 May 21. PMID: 24850756; PMCID: PMC4110656.

Francklyn MM, Borgatia AD, Elombila M, Reddy AG, Tareq K. Retroperitoneal fibrosis. Exceptional cause of renal artery thrombosis with secondary hypertension. (2013) A case from University Mohamed V, CheikhZaid Hospital, Rabat, March 2013. J Cardiol Cases. Dec; 13;9(1):26-28. doi: 10.1016/j.jccase.2013.09.006. PMID: 30546777; PMCID: PMC6281554.

Greco P, Vaglio A, Corradi D, Cobelli R, Zompatori M, Buzio C. (2005) Tuberculosis as a trigger of retroperitoneal fibrosis. Clin Infect Dis. Oct ;1;41(7):e72-5. doi: 10.1086/433189. Epub 2005 Aug 31. PMID: 16142654.

Hedgire SS, McDermott S, Borczuk D, Elmi A, Saini S, Harisinghani MG. (2013) The spectrum of IgG4-related disease in the abdomen and pelvis. AJR Am J Roentgenol. Jul;201(1):14-22. doi: 10.2214/AJR.12.9995. PMID: 23789654.

Jadhav KK, Kumar V, Punatar CB, Joshi VS, Sagade SN. (2017) Retroperitoneal fibrosis-clinical presentation and outcome analysis from urological perspective. Investig Clin Urol. Sep;58(5):371-377. doi: 10.4111/icu.2017.58.5.371. Epub 2017 Aug 4. PMID: 28868510; PMCID: PMC5577335.

Jansen I, Hendriksz TR, Van Bommel EF. Pseudocyst formation in retroperitoneal fibrosis relapse. (2010) Br J RadiolJun;83(990):e111-3. doi: 10.1259/bjr/81915147. PMID: 20505023; PMCID: PMC3473591.

Jois RN, Kerrigan N, Scott DG. (2007) Mycophenolate mofetil for maintenance of remission in idiopathic retroperitoneal fibrosis. Rheumatology (Oxford).; Apr ;46(4):717-8. doi: 10.1093/rheumatology/kel417. Epub 2007 Jan 11. PMID: 17218329.

Jun BM, Lee EY, Yoon YJ, Kim EK, Ahn MS, Lee CK, Cho YS, Yoo B, Moon HB. (2001) Retroperitoneal fibrosis with duodenal stenosis. J Korean Med Sci. Jun;16(3):371-4. doi: 10.3346/jkms.2001.16.3.371. PMID: 11410704; PMCID: PMC3054755.

Kaddouri Said, Ghoundale Omar, Chahbi Zakaria, Essadi Ismail, Qacif Hassan, Zamraoui Nadir, Zyani Mohamed. (2017) Retroperitoneal Fibrosis: Case Series of 20 Patients, Science Journal of Public Health. Vol. 5, No. 4, pp. 294-300. doi: 10.11648/j.sjph.20170504.13.

Kermani TA, Crowson CS, Achenbach SJ, Luthra HS. (2011) sIdiopathic retroperitoneal fibrosis: a retrospective review of clinical presentation, treatment, and outcomes. Mayo Clin Proc. Apr ;86(4):297-303. doi: 10.4065/mcp.2010.0663. PMID: 21454732; PMCID: PMC3068889.

Khosroshahi A, Carruthers MN, Stone JH, Shinagare S, Sainani N, Hasserjian RP, Deshpande V. (2013) Rethinking Ormond's disease: "idiopathic" retroperitoneal fibrosis in the era of IgG4-related disease. Medicine (Baltimore). Mar;92(2):82-91. doi: 10.1097/MD.0b013e318289610f. PMID: 23429355; PMCID: PMC4553983.

Kirby DT, Dilday JC, Muir KB, Young A, and Aidinian G. (2020) Inferior vena cava occlusion in retroperitoneal fibrosis andexogenous testosterone administration; Journal of Vascular Surgery Cases and Innovative Techniques. Vol 6, Number 1; 34-37.

Laroche AS, Bell RZ, Bezzaoucha S, Földes E, Lamarche C, Vallée M. (2016) Retroperitoneal fibrosis: retrospective descriptive study on clinical features and management. Res Rep Urol. Oct ;26;8:175-179. doi: 10.2147/RRU.S115448. PMID: 27822461; PMCID: PMC5087765.

Magrey MN, Husni ME, Kushner I, Calabrese LH. (2009) Do acute-phase reactants predict response to glucocorticoid therapy in retroperitoneal fibrosis? Arthritis Rheum. May ;15;61(5):674-9. doi: 10.1002/art.24461. PMID: 19404998.

Mangieri D, Corradi D, Martorana D, Malerba G, Palmisano A, Libri I, Bartoli V, Carnevali ML, Goldoni M, Govoni P, Alinovi R, Buzio C, Vaglio A. (2012) Eotaxin/CCL11 in idiopathic retroperitoneal fibrosis. Nephrol Dial Transplant. Oct;27(10):3875-84. doi: 10.1093/ndt/gfs408. PMID: 23114905.

Meier P, Vogt B, Blanc E. (2007) Rethinking the triggering inflammatory processes of chronic periaortitis. Nephron Exp Nephrol. 105(1): e17-23. doi: 10.1159/000097015. Epub 2006 Nov 13. PMID: 17108706.

Mertens S, Zeegers AG, Wertheimer PA, Hendriksz TR, van Bommel EF. (2014) Efficacy and complications of urinary drainage procedures in idiopathic retroperitoneal fibrosis complicated by extrinsic ureteral obstruction. Int J Urol. Mar;21(3):283-8. doi: 10.1111/iju.12234. Epub 2013 Aug 22. PMID: 24033464.

Mirault T, Lambert M, Puech P, Argatu D, Renaud A, Duhamel A, Glovacki F, Villers A, Hachulla E, Biserte J, Hatron PY, Lemaitre L. (2012) Malignant retroperitoneal fibrosis: MRI characteristics in 50 patients. Medicine (Baltimore). Sep;91(5):242-50. doi: 10.1097/MD.0b013e31826b1c1d. PMID: 22932788.

Mullineux JH, Ivan CV, Pancholi J, Verma R, Rajesh A, Verma S, Stephenson JA. (2018) Benign Sclerosing and Fibrosing Conditions of the Abdomen and Their Potential Mimics. J Clin Imaging Sci. May ;31;8:21. doi: 10.4103/jcis.JCIS_19_18. PMID: 29963328; PMCID: PMC5998607.

Murakami N, Arai Y, Takagawa Y, Okuma K, Takahashi K, Inaba K, Igaki H, Nakayama Y, Itami J. (2018) Inferior vena cava syndrome caused by retroperitoneal fibrosis after pelvic irradiation: A case report. Gynecol Oncol Rep. Dec ;4;27:19-21. doi: 10.1016/j.gore.2018.12.002. PMID: 30560192; PMCID: PMC6289939.

Palmisano A, Urban ML, Corradi D, Cobelli R, Alberici F, Maritati F, Versari A, Pipitone N, Salvarani C, Buzio C, Vaglio A. (2015) Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? Rheumatology (Oxford). Nov;54(11):2004-9. doi: 10.1093/rheumatology/kev225. Epub 2015 Jun 23. PMID: 26106209.

Pelkmans LG, Aarnoudse AJ, Hendriksz TR, van Bommel EF. (2012) Value of acute-phase reactants in monitoring disease activity and treatment response in idiopathic retroperitoneal fibrosis. Nephrol Dial Transplant. Jul;27(7):2819-25. doi: 10.1093/ndt/gfr779. Epub 2012 Jan 23. PMID: 22273666.

Ramshaw AL, Roskell DE, Parums DV. (1994) Cytokine gene expression in aortic adventitial inflammation associated with advanced atherosclerosis (chronic periaortitis). J Clin Pathol. Aug;47(8):721-7. doi: 10.1136/jcp.47.8.721. PMID: 7962625; PMCID: PMC502145.

Rosenkrantz AB, Spieler B, Seuss CR, Stifelman MD, Kim S. (2012) Utility of MRI features for differentiation of retroperitoneal fibrosis and lymphoma. AJR Am J Roentgenol. Jul;199(1):118-26. doi: 10.2214/AJR.11.7822. PMID: 22733902.

Salvarani C, Calamia KT, Matteson EL, Hunder GG, Pipitone N, Miller DV, Warrington KJ. (2011) Vasculitis of the gastrointestinal tract in chronic periaortitis. Medicine (Baltimore). Jan;90(1):28-39. doi: 10.1097/MD.0b013e318207231e. PMID: 21200184.

Scheel PJ Jr, Feeley N. (2009) Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. Medicine (Baltimore). Jul ;88(4):202-7. doi: 10.1097/MD.0b013e3181afc439. PMID: 19593224.

Scheel PJ Jr, Feeley N. (2013) Retroperitoneal fibrosis. Rheum Dis Clin North Am. May;39(2):365-81. doi: 10.1016/j.rdc.2013.02.004. Epub 2013 Mar 9. PMID: 23597969.

Scheel PJ Jr, Sozio SM, Feeley N. (2012) Medical management of retroperitoneal fibrosis. Trans Am Clin Climatol Assoc. vol.123:283-90; discussion 290-1. PMID: 23303996; PMCID: PMC3540636.

Sili Scavalli A, Di Rocco G, Giannotti D, Patrizi G, Frezzotti F, Bernieri MG, Cantisani V, Redler A. (2012) Two-years follow-up of low-dose methotrexate and 6-methylprednisolone therapy in a patient with idiopathic retroperitoneal fibrosis. Eur Rev Med Pharmacol Sci. Dec ;16(15):2171-4. PMID: 23280036.

Subramani AV, Lockwood GM, Jetton JG, Dhungana N, Sato TS. (2019) Pediatric idiopathic retroperitoneal fibrosis. Radiol Case Rep. Jan ;31;14(4):459-462. doi: 10.1016/j.radcr.2019.01.006. PMID: 30740191; PMCID: PMC6357545.

Tanaka T, Masumori N. (2020) Current approach to diagnosis and management of retroperitoneal fibrosis. Int J Urol. May;27(5):387-394. doi: 10.1111/iju.14218. Epub 2020 Mar 12. PMID: 32166828.

Tanuma Y, Yokoo A. (2002) [Idiopathic retroperitoneal fibrosis with large vessel thrombosis]. Hinyokika Kiyo. Sep;48(9):539-43. Japanese. PMID: 12402479.

Treglia G, Mattoli MV, Bertagna F. (2012) Fluorine-18-fluorodeoxyglucose positron emission tomography in assessing retroperitoneal fibrosis: a literature review. Int J Mol Imaging.;2012:484052. doi: 10.1155/2012/484052. Epub 2012 Sep 25. PMID: 23050140; PMCID: PMC3461285.

Uibu T, Järvenpää R, Hakomäki J, Auvinen A, Honkanen E, Metsärinne K, Roto P, Saha H, Uitti J, Oksa P. (2008) Asbestos-related pleural and lung fibrosis in patients with retroperitoneal fibrosis. Orphanet J Rare Dis. Nov; 13;3:29. doi: 10.1186/1750-1172-3-29. PMID: 19014533; PMCID: PMC2596089.

Vaglio A, Manenti L, Allegri L, Ferrozzi F, Corradi D, Buzio C. (2002) ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis? J Intern Med. Mar;251(3):268-71. doi: 10.1046/j.1365-2796.2002.00948.x. PMID: 11886487.

Vaglio A, Versari A, Fraternali A, Ferrozzi F, Salvarani C, Buzio C. (2005) (18)F-fluorodeoxyglucose positron emission tomography in the diagnosis and followup of idiopathic retroperitoneal fibrosis. Arthritis Rheum. Feb ;15;53(1):122-5. doi: 10.1002/art.20914. PMID: 15696564.

Vaglio A. & Maritati F. (2016) Idiopathic Retroperitoneal Fibrosis. J AmSoc Nephrol;27: 1880–1889.

van Bommel EF, Jansen I, Hendriksz TR, Aarnoudse AL. (2009) Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore). Jul;88(4):193-201. doi: 10.1097/MD.0b013e3181afc420. PMID: 19593223.

Yilmaz M, Akbulut S, Samdanci ET, Yilmaz S. (2012) Abdominopelvic actinomycosis associated with an intrauterine device and presenting with a rectal mass and hydronephrosis: a troublesome condition for the clinician. Int Surg. Jul-Sep;97(3):254-9. doi: 10.9738/CC121.1. PMID: 23113856; PMCID: PMC3723221.

Zen Y, Onodera M, Inoue D, Kitao A, Matsui O, Nohara T, Namiki M, Kasashima S, Kawashima A, Matsumoto Y, Katayanagi K, Murata T, Ishizawa S, Hosaka N, Kuriki K, Nakanuma Y. (2009) Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol. Dec;33(12):1833-9. doi: 10.1097/pas.0b013e3181b72882. PMID: 19950407.

Zhang J, Chen H, Ma Y, Xiao Y, Niu N, Lin W, Wang X, Liang Z, Zhang F, Li F, Zhang W, Zhu Z. (2014) Characterizing IgG4-related disease with ¹⁸F-FDG PET/CT: a prospective cohort study. Eur J Nucl Med Mol Imaging. Aug;41(8):1624-34. doi: 10.1007/s00259-014-2729-3. Epub 2014 Apr 25. PMID: 24764034; PMCID: PMC4089015.

Zhang W, Xue F, Wang C, Shao L. (2017) Clinical features and prognostic factors of ten patients with renal failure caused by IgG4-related retroperitoneal fibrosis. Oncotarget. Dec ;7;9(2):2858-2865. doi: 10.18632/oncotarget.23103. PMID: 29416818; PMCID: PMC5788686.

Zhou HJ, Yan Y, Zhou B, Lan TF, Wang XY, Li CS. Retroperitoneal fibrosis. (2015) A Retrospective clinical data analysis of 30 patients in a 10-year period. Chin Med J (Engl). Mar ;20;128(6):804-10. doi: 10.4103/0366-6999.152648. PMID: 25758277; PMCID: PMC4833987