Treatment of retroperitoneal fibrosis
- Augusto Vaglio, MD, PhD
Augusto Vaglio, MD, PhD
- University Hospital, Parma, Italy
- Alessandra Palmisano, MD, PhD
Alessandra Palmisano, MD, PhD
- Department of Clinical and Experimental Medicine
- University of Parma, Italy
Retroperitoneal fibrosis, also referred to as Ormond's disease, is an uncommon but treatable cause of obstructive uropathy. This disorder involves chronic inflammation and fibroblast proliferation, with excessive extracellular matrix deposition. Untreated patients may develop severe complications or progress to end-stage renal disease (ESRD) .
Retroperitoneal fibrosis is also sometimes associated with venous or arterial occlusion due to compression or abdominal aortic aneurysms, if in the setting of chronic periaortitis. In addition to medical therapy for the retroperitoneal fibrosis, these situations may require endovascular or surgical management. The management of these issues requires consultation with the appropriate specialists. (See "Clinical manifestations and diagnosis of retroperitoneal fibrosis".)
The treatment of idiopathic retroperitoneal fibrosis is reviewed here. The clinical manifestations, pathogenesis, and diagnosis of this disorder are presented separately. (See "Clinical manifestations and diagnosis of retroperitoneal fibrosis".)
GENERAL APPROACH TO THERAPY
The goals of therapy are to relieve the obstruction caused by fibrosis, stop the progression of the fibrotic process, and prevent recurrence.
If the retroperitoneal fibrosis is due to a secondary cause (table 1), treatment is aimed at the underlying etiology. If the disease is idiopathic, we initiate immunosuppressive therapy.To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
- Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367:241.
- Harreby M, Bilde T, Helin P, et al. Retroperitoneal fibrosis treated with methylprednisolon pulse and disease-modifying antirheumatic drugs. Scand J Urol Nephrol 1994; 28:237.
- Cogan E, Fastrez R. Azathioprine. An alternative treatment for recurrent idiopathic retroperitoneal fibrosis. Arch Intern Med 1985; 145:753.
- Marcolongo R, Tavolini IM, Laveder F, et al. Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 2004; 116:194.
- Jois RN, Gaffney K, Marshall T, Scott DG. Chronic periaortitis. Rheumatology (Oxford) 2004; 43:1441.
- van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002; 60:231.
- Ross JC, Goldsmith HJ. The combined surgical and medical treatment of retroperitoneal fibrosis. Br J Surg 1971; 58:422.
- Mertens S, Zeegers AG, Wertheimer PA, et al. Efficacy and complications of urinary drainage procedures in idiopathic retroperitoneal fibrosis complicated by extrinsic ureteral obstruction. Int J Urol 2014; 21:283.
- Srinivasan AK, Richstone L, Permpongkosol S, Kavoussi LR. Comparison of laparoscopic with open approach for ureterolysis in patients with retroperitoneal fibrosis. J Urol 2008; 179:1875.
- Tiptaft RC, Costello AJ, Paris AM, Blandy JP. The long-term follow-up of idiopathic retroperitoneal fibrosis. Br J Urol 1982; 54:620.
- Zhang QX, Magovern CJ, Mack CA, et al. Vascular endothelial growth factor is the major angiogenic factor in omentum: mechanism of the omentum-mediated angiogenesis. J Surg Res 1997; 67:147.
- Lodde M, Pycha A, Palermo S, et al. Uretero-ureterocutaneostomy (wrapped by omentum). BJU Int 2005; 95:371.
- Vaglio A, Palmisano A, Alberici F, et al. Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 2011; 378:338.
- van Bommel EF, van Spengler J, van der Hoven B, Kramer P. Retroperitoneal fibrosis: report of 12 cases and a review of the literature. Neth J Med 1991; 39:338.
- van Bommel EF, Siemes C, Hak LE, et al. Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis 2007; 49:615.
- Kardar AH, Kattan S, Lindstedt E, Hanash K. Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol 2002; 168:550.
- Resnick MI, Kursh ED. Extrinsic obstruction of the ureter. In: Campbell's Urology, 6th ed., WB Saunders, Philadelphia 1992. p.514.
- Fernando A, Pattison J, Horsfield C, et al. [(18)F]-Fluorodeoxyglucose Positron Emission Tomography in the Diagnosis, Treatment Stratification, and Monitoring of Patients with Retroperitoneal Fibrosis: A Prospective Clinical Study. Eur Urol 2017; 71:926.
- Magrey MN, Husni ME, Kushner I, Calabrese LH. Do acute-phase reactants predict response to glucocorticoid therapy in retroperitoneal fibrosis? Arthritis Rheum 2009; 61:674.
- Grotz W, von Zedtwitz I, Andre M, Schollmeyer P. Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. Lancet 1998; 352:1195.
- Jois RN, Kerrigan N, Scott DG. Mycophenolate mofetil for maintenance of remission in idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 2007; 46:717.
- Marzano A, Trapani A, Leone N, et al. Treatment of idiopathic retroperitoneal fibrosis using cyclosporin. Ann Rheum Dis 2001; 60:427.
- Al-Harthy F, Esdaile J, Berean KW, Chalmers A. Multifocal idiopathic fibrosclerosis: treatment of 2 cases with cyclosporine. J Rheumatol 2006; 33:358.
- Scavalli AS, Spadaro A, Riccieri V, et al. Long-term follow-up of low-dose methotrexate therapy in one case of idiopathic retroperitoneal fibrosis. Clin Rheumatol 1995; 14:481.
- Scheel PJ Jr, Piccini J, Rahman MH, et al. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis. J Urol 2007; 178:140.
- Scheel PJ Jr, Feeley N, Sozio SM. Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series. Ann Intern Med 2011; 154:31.
- Bourouma R, Chevet D, Michel F, et al. Treatment of idiopathic retroperitoneal fibrosis with tamoxifen. Nephrol Dial Transplant 1997; 12:2407.
- Clark CP, Vanderpool D, Preskitt JT. The response of retroperitoneal fibrosis to tamoxifen. Surgery 1991; 109:502.
- Ozener C, Kiriş S, Lawrence R, et al. Potential beneficial effect of tamoxifen in retroperitoneal fibrosis. Nephrol Dial Transplant 1997; 12:2166.
- Tziomalos K, Krikis N, Karagiannis A, et al. Treatment of idiopathic retroperitoneal fibrosis with combined administration of corticosteroids and tamoxifen. Clin Nephrol 2004; 62:74.
- van Bommel EF, Hendriksz TR, Huiskes AW, Zeegers AG. Brief communication: tamoxifen therapy for nonmalignant retroperitoneal fibrosis. Ann Intern Med 2006; 144:101.
- Moroni G, Gallelli B, Banfi G, et al. Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches. Nephrol Dial Transplant 2006; 21:2485.
- Costanzi S, Zoli A, Ferraro PM, et al. A paraneoplastic retroperitoneal fibrosis resistant to corticosteroids treated with tamoxifen. Clin Nephrol 2008; 70:172.
- van Bommel EF, Pelkmans LG, van Damme H, Hendriksz TR. Long-term safety and efficacy of a tamoxifen-based treatment strategy for idiopathic retroperitoneal fibrosis. Eur J Intern Med 2013; 24:444.
- Vega J, Goecke H, Tapia H, et al. Treatment of idiopathic retroperitoneal fibrosis with colchicine and steroids: a case series. Am J Kidney Dis 2009; 53:628.
- Catanoso MG, Spaggiari L, Magnani L, et al. Efficacy of infliximab in a patient with refractory idiopathic retroperitoneal fibrosis. Clin Exp Rheumatol 2012; 30:776.
- Couderc M, Mathieu S, Dubost JJ, Soubrier M. Retroperitoneal fibrosis during etanercept therapy for rheumatoid arthritis. J Rheumatol 2013; 40:1931.
- Vaglio A, Catanoso MG, Spaggiari L, et al. Interleukin-6 as an inflammatory mediator and target of therapy in chronic periaortitis. Arthritis Rheum 2013; 65:2469.
- Maritati F, Corradi D, Versari A, et al. Rituximab therapy for chronic periaortitis. Ann Rheum Dis 2012; 71:1262.
- Perrotta FM, Fici M, Guerra G, et al. Chronic periaortitis with retroperitoneal fibrosis successfully treated with first line tocilizumab monotherapy: a case report. Clin Exp Rheumatol 2017; 35 Suppl 103:226.
- Vaglio A, Greco P, Versari A, et al. Post-treatment residual tissue in idiopathic retroperitoneal fibrosis: active residual disease or silent "scar" ? A study using 18F-fluorodeoxyglucose positron emission tomography. Clin Exp Rheumatol 2005; 23:231.
- Heidenreich A, Derakhshani P, Neubauer S, Krug B. [Treatment outcomes in primary and secondary retroperitoneal fibrosis]. Urologe A 2000; 39:141.
- Alexopoulos E, Memmos D, Bakatselos S, et al. Idiopathic retroperitoneal fibrosis: a long-term follow-up study. Eur Urol 1987; 13:313.
- Alberici F, Palmisano A, Urban ML, et al. Methotrexate plus prednisone in patients with relapsing idiopathic retroperitoneal fibrosis. Ann Rheum Dis 2013; 72:1584.
- Baker LR, Mallinson WJ, Gregory MC, et al. Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol 1987; 60:497.
- GENERAL APPROACH TO THERAPY
- INTERVENTION IN PATIENTS WITH URINARY TRACT OBSTRUCTION
- Selection of an interventional procedure
- Monitoring the response to interventional therapy
- MEDICAL THERAPY
- Medical treatment of secondary disease
- Medical treatment of idiopathic disease
- - Initial therapy with glucocorticoids
- Glucocorticoid-resistant disease
- - Initial therapy with tamoxifen in patients who cannot take glucocorticoids
- - Duration of therapy
- - Alternative initial therapy with combined immunosuppression
- - Investigative therapies
- Monitoring the response to medical therapy
- Recurrence after discontinuation of therapy
- SOCIETY GUIDELINE LINKS
- SUMMARY AND RECOMMENDATIONS