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Clinical manifestations and diagnosis of retroperitoneal fibrosis

Augusto Vaglio, MD, PhD
Alessandra Palmisano, MD, PhD
Section Editor
Gary C Curhan, MD, ScD
Deputy Editor
Alice M Sheridan, MD


Retroperitoneal fibrosis is a rare condition characterized by the presence of inflammatory and fibrous retroperitoneal tissue that often encases the ureters or abdominal organs [1]. This disorder was initially called Ormond's disease [2], but it has also been referred to as periureteritis fibrosa, periureteritis plastica, chronic periureteritis, sclerosing retroperitoneal granuloma, and fibrous retroperitonitis.

Retroperitoneal fibrosis may be idiopathic or secondary to other causes. Idiopathic retroperitoneal fibrosis is an immune-mediated disease, which can be either isolated, associated with other autoimmune diseases, or arise in the context of a multifocal fibroinflammatory disorder, named immunoglobulin G4-related disease (IgG4-RD). Although there are no standardized criteria of classification, idiopathic retroperitoneal fibrosis is actually part of the disease spectrum of chronic periaortitis, a condition characterized by inflammation and fibrosis surrounding the aorta and iliac arteries [1,3,4]. Chronic periaortitis (CP) includes inflammatory abdominal aortic aneurysms and perianeurysmal retroperitoneal fibrosis, two entities that are grouped together because of similar clinical and histologic characteristics, although pathogeneses and epidemiology may differ [5]. In approximately one-third of patients, CP also involves the thoracic aorta and the epiaortic arteries, suggesting that it is a systemic inflammatory disease of the large arteries [6].

The etiology, pathogenesis, clinical manifestations, and diagnostic evaluation of retroperitoneal fibrosis will be reviewed here. The treatment of retroperitoneal fibrosis is presented separately (see "Treatment of retroperitoneal fibrosis"). Other causes of urinary tract obstruction and hydronephrosis are also reviewed elsewhere. (See "Clinical manifestations and diagnosis of urinary tract obstruction and hydronephrosis".)


Retroperitoneal fibrosis is a rare disease. In one Finnish study, the incidence of the idiopathic form of the disease was estimated from hospital discharge data to be 0.1 per 100,000 person-years and its prevalence 1.4 per 100,000 inhabitants [7]. However, in a subsequent population-based study performed in the Netherlands, the reported annual incidence was approximately 10-fold higher (ie, 1.3 per 100,000 inhabitants per year) [8]. The latter study probably provides a more accurate estimate of the incidence given the complexity of the diagnosis and requirement for prolonged follow-up that would not have been possible in a study of hospital discharge codes [9]. Inflammatory abdominal aortic aneurysms represent 4 to 10 percent of all abdominal aortic aneurysms [10]. No data are available about the incidence of secondary retroperitoneal fibrosis.

Idiopathic disease most commonly occurs in individuals 40 to 60 years of age [5,8,11,12]. Most studies have suggested a 2 to 3:1 male-to-female predominance [5,8,11,12], although this is not reported in all studies [4]. One study has suggested that chronic periaortitis, which includes idiopathic retroperitoneal fibrosis, is associated with the human leukocyte antigen (HLA) allele HLA-DRB1*03 [13].

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Literature review current through: Sep 2017. | This topic last updated: Sep 25, 2017.
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  1. Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367:241.
  2. ORMOND JK. Bilateral ureteral obstruction due to envelopment and compression by an inflammatory retroperitoneal process. J Urol 1948; 59:1072.
  3. van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002; 60:231.
  4. Scheel PJ Jr, Feeley N. Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. Medicine (Baltimore) 2009; 88:202.
  5. 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.
  6. Palmisano A, Urban ML, Corradi D, et al. Chronic periaortitis with thoracic aorta and epiaortic artery involvement: a systemic large vessel vasculitis? Rheumatology (Oxford) 2015; 54:2004.
  7. Uibu T, Oksa P, Auvinen A, et al. Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 2004; 363:1422.
  8. van Bommel EF, Jansen I, Hendriksz TR, Aarnoudse AL. Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore) 2009; 88:193.
  9. Vaglio A. Retroperitoneal fibrosis: new insights into clinical presentation and diagnosis. Medicine (Baltimore) 2009; 88:208.
  10. Rasmussen TE, Hallett JW Jr. Inflammatory aortic aneurysms. A clinical review with new perspectives in pathogenesis. Ann Surg 1997; 225:155.
  11. Koep L, Zuidema GD. The clinical significance of retroperitoneal fibrosis. Surgery 1977; 81:250.
  12. Kasales CJ, Hartman DS. Genitourinary case of the day. Retroperitoneal fibrosis. AJR Am J Roentgenol 1994; 162:1454.
  13. Martorana D, Vaglio A, Greco P, et al. Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin. Arthritis Rheum 2006; 55:126.
  14. Cronin CG, Lohan DG, Blake MA, et al. Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol 2008; 191:423.
  15. Graham JR, Suby HI, LeCompte PR, Sadowsky NL. Fibrotic disorders associated with methysergide therapy for headache. N Engl J Med 1966; 274:359.
  17. Goenka AH, Shah SN, Remer EM. Imaging of the retroperitoneum. Radiol Clin North Am 2012; 50:333.
  18. Goldoni M, Bonini S, Urban ML, et al. Asbestos and smoking as risk factors for idiopathic retroperitoneal fibrosis: a case-control study. Ann Intern Med 2014; 161:181.
  19. Couderc M, Mathieu S, Dubost JJ, Soubrier M. Retroperitoneal fibrosis during etanercept therapy for rheumatoid arthritis. J Rheumatol 2013; 40:1931.
  20. Hosaka N, Ito M, Taki Y, et al. Amyloid A gastrointestinal amyloidosis associated with idiopathic retroperitoneal fibrosis. Report of a rare autopsy case and review of the literature. Arch Pathol Lab Med 2003; 127:735.
  21. Jendro MC, Zeidler H, Rosenthal H, et al. Improvement of Erdheim-Chester disease in two patients by sequential treatment with vinblastine and mycophenolate mofetil. Clin Rheumatol 2004; 23:52.
  22. Janssen T, van Cangh PJ. [Retroperitoneal fibrosis due to barium]. Prog Urol 1994; 4:429.
  23. Remmele W, Müller-Lobeck H, Paulus W. Primary mesenteritis, mesenteric fibrosis and mesenteric fibromatosis. Report of four cases, pathology, and classification. Pathol Res Pract 1988; 184:77.
  24. Nesti M, Marinaccio A, Chellini E. Malignant mesothelioma in Italy, 1997. Am J Ind Med 2004; 45:55.
  25. Salvarani C, Calamia KT, Matteson EL, et al. Vasculitis of the gastrointestinal tract in chronic periaortitis. Medicine (Baltimore) 2011; 90:28.
  26. Hamano H, Kawa S, Ochi Y, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 2002; 359:1403.
  27. Boiardi L, Vaglio A, Nicoli D, et al. CC chemokine receptor 5 polymorphism in chronic periaortitis. Rheumatology (Oxford) 2011; 50:1025.
  28. Mangieri D, Corradi D, Martorana D, et al. Eotaxin/CCL11 in idiopathic retroperitoneal fibrosis. Nephrol Dial Transplant 2012; 27:3875.
  29. Neild GH, Rodriguez-Justo M, Wall C, Connolly JO. Hyper-IgG4 disease: report and characterisation of a new disease. BMC Med 2006; 4:23.
  30. Zen Y, Onodera M, Inoue D, et al. Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 2009; 33:1833.
  31. Zen Y, Sawazaki A, Miyayama S, et al. A case of retroperitoneal and mediastinal fibrosis exhibiting elevated levels of IgG4 in the absence of sclerosing pancreatitis (autoimmune pancreatitis). Hum Pathol 2006; 37:239.
  32. Stone JR. Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol 2011; 23:88.
  33. Castelein T, Coudyzer W, Blockmans D. IgG4-related periaortitis vs idiopathic periaortitis: is there a role for atherosclerotic plaque in the pathogenesis of IgG4-related periaortitis? Rheumatology (Oxford) 2015; 54:1250.
  34. Rossi GM, Rocco R, Accorsi Buttini E, et al. Idiopathic retroperitoneal fibrosis and its overlap with IgG4-related disease. Intern Emerg Med 2017; 12:287.
  35. Witten DM. Retroperitoneal fibrosis. In: Clinical Urography, Pollack HM (Ed), WB Saunders, Philadelphia 1990. p.2469.
  36. Demko TM, Diamond JR, Groff J. Obstructive nephropathy as a result of retroperitoneal fibrosis: a review of its pathogenesis and associations. J Am Soc Nephrol 1997; 8:684.
  37. Littlejohn GO, Keystone EC. The association of retroperitoneal fibrosis with systemic vasculitis and HLA-B27: a case report and review of the literature. J Rheumatol 1981; 8:665.
  39. Saxton HM, Kilpatrick FR, Kinder CH, et al. Retroperitoneal fibrosis. A radiological and follow-up study of fourteen cases. Q J Med 1969; 38:159.
  40. 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.
  41. Baker LR, Mallinson WJ, Gregory MC, et al. Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol 1987; 60:497.
  42. Warnatz K, Keskin AG, Uhl M, et al. Immunosuppressive treatment of chronic periaortitis: a retrospective study of 20 patients with chronic periaortitis and a review of the literature. Ann Rheum Dis 2005; 64:828.
  43. Ceresini G, Urban ML, Corradi D, et al. Association between idiopathic retroperitoneal fibrosis and autoimmune thyroiditis: a case-control study. Autoimmun Rev 2015; 14:16.
  44. Oshiro H, Ebihara Y, Serizawa H, et al. Idiopathic retroperitoneal fibrosis associated with immunohematological abnormalities. Am J Med 2005; 118:782.
  45. Fagan CJ, Larrieu AJ, Amparo EG. Retroperitoneal fibrosis: ultrasound and CT features. AJR Am J Roentgenol 1979; 133:239.
  46. Feinstein RS, Gatewood OM, Goldman SM, et al. Computerized tomography in the diagnosis of retroperitoneal fibrosis. J Urol 1981; 126:255.
  47. Jois RN, Gaffney K, Marshall T, Scott DG. Chronic periaortitis. Rheumatology (Oxford) 2004; 43:1441.
  48. Dalla-Palma L, Rocca-Rossetti S, Pozzi-Mucelli RS, Rizzatto G. Computed tomography in the diagnosis of retroperitoneal fibrosis. Urol Radiol 1981; 3:77.
  49. George V, Tammisetti VS, Surabhi VR, Shanbhogue AK. Chronic fibrosing conditions in abdominal imaging. Radiographics 2013; 33:1053.
  50. Amis ES Jr. Retroperitoneal fibrosis. AJR Am J Roentgenol 1991; 157:321.
  51. Arrivé L, Hricak H, Tavares NJ, Miller TR. Malignant versus nonmalignant retroperitoneal fibrosis: differentiation with MR imaging. Radiology 1989; 172:139.
  52. Bakir B, Yilmaz F, Turkay R, et al. Role of diffusion-weighted MR imaging in the differentiation of benign retroperitoneal fibrosis from malignant neoplasm: preliminary study. Radiology 2014; 272:438.
  53. Corradi D, Maestri R, Palmisano A, et al. Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int 2007; 72:742.
  54. Persky L, Huus JC. Atypical manifestations of retroperitoneal fibrosis. J Urol 1974; 111:340.
  55. Saldino RM, Palubinskas AJ. Medial placement of the ureter: a normal variant which may simulate retroperitoneal fibrosis. J Urol 1972; 107:582.
  56. Emley TE, Cain MP, Faught PR, Davis MM. Ureteropelvic junction obstruction due to inflammatory pseudotumor masquerading as hydronephrosis because of a neuropathic bladder in a child with myelomeningocele. Urology 2001; 58:105.
  57. Stoeckle E, Coindre JM, Bonvalot S, et al. Prognostic factors in retroperitoneal sarcoma: a multivariate analysis of a series of 165 patients of the French Cancer Center Federation Sarcoma Group. Cancer 2001; 92:359.
  58. Mirault T, Lambert M, Puech P, et al. Malignant retroperitoneal fibrosis: MRI characteristics in 50 patients. Medicine (Baltimore) 2012; 91:242.
  59. Palmisano A, Vaglio A. Chronic periaortitis: a fibro-inflammatory disorder. Best Pract Res Clin Rheumatol 2009; 23:339.
  60. Vaglio A, Corradi D, Manenti L, et al. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 2003; 114:454.
  61. Moroni G, Castellani M, Balzani A, et al. The value of (18)F-FDG PET/CT in the assessment of active idiopathic retroperitoneal fibrosis. Eur J Nucl Med Mol Imaging 2012; 39:1635.
  62. Fendler WP, Eiber M, Stief CG, Herrmann K. A PET for All Seasons: (18)F-Fluorodeoxyglucose to Characterize Inflammation and Malignancy in Retroperitoneal Fibrosis? Eur Urol 2017; 71:934.