Download PDF
Rofo 2020; 192(10): 929-936
DOI: 10.1055/a-1181-9205
Review

Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging

Article in several languages: English | deutsch
Felix Peisen
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Wolfgang Maximilian Thaiss
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Kaspar Ekert
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Marius Horger
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Bastian Amend
2   Department of Urology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Jens Bedke
2   Department of Urology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Konstantin Nikolaou
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
,
Sascha Kaufmann
1   Department of Diagnostic and Interventional Radiology, Eberhard Karls University Tübingen, Tübingen, Germany
› Author Affiliations
› Further Information
Also available at
   Permissions and Reprints

Abstract

Background Retroperitoneal fibrosis is a rare disease with an incidence of 0–1/100 000 inhabitants per year and is associated with chronic inflammatory fibrosis of the retroperitoneum and the abdominal aorta. This article sheds light on the role of radiological imaging in retroperitoneal fibrosis, names various differential diagnoses and provides an overview of drug and surgical treatment options.

Methods A literature search for the keywords “retroperitoneal fibrosis” and “Ormond’s disease” was carried out in the PubMed database between January 1, 1995 and December 31, 2019 (n = 1806). Mainly original papers were selected, but also reviews, in English and German language, with a focus on publications in the last 10 years, without excluding older publications that the authors believe are relevant to the topic discussed in the review (n = 40).

Results and Conclusion Ormond’s disease is a rare but important differential diagnosis for nonspecific back and flank pain. Imaging diagnostics using CT or MRI show a retroperitoneal mass, which must be differentiated from lymphoma, sarcoma, multiple myeloma and Erdheim-Chester disease. Patients have an excellent prognosis under adequate therapy. FDG-PET/CT or FDG-PET/MRT should be considered as potential modalities, as hybrid imaging can evaluate both the morphological changes and the inflammation.

Key Points: 

  • Ormond’s disease is a differential diagnosis for nonspecific back and flank pain.

  • Radiological imaging is essential and the gold standard in the diagnosis and follow-up of RPF.

  • Patients have an excellent prognosis under adequate therapy.

Citation Format

  • Peisen F, Thaiss WM, Ekert K et al. Retroperitoneal Fibrosis and its Differential Diagnoses: The Role of Radiological Imaging. Fortschr Röntgenstr 2020; 192: 929 – 936

Key words

retroperitoneum - urinary - fibrosis, cystic - inflammation


Publication History

Received: 02 January 2020

Accepted: 24 April 2020

Article published online:
22 July 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 

  • References

  • 1 Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241-251 . doi:10.1016/S0140-6736(06)68035-5
    CrossrefPubMedGoogle Scholar
  • 2 Vogt B, Meier P, Burnier M. Retroperitoneal fibrosis, M. Ormond, periaoartitis, ...?. Ther Umsch 2008; 65: 265-268 . doi:10.1024/0040-5930.65.5.265
    CrossrefPubMedGoogle Scholar
  • 3 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: 926-933 . doi:10.1016/j.eururo.2016.10.046
    CrossrefPubMedGoogle Scholar
  • 4 Swartz RD. Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment. Am J Kidney Dis 2009; 54: 546-553 . doi:10.1053/j.ajkd.2009.04.019
    CrossrefPubMedGoogle Scholar
  • 5 Chandna A, Sharma AP, Pareek T. et al IgG4-related Retroperitoneal Fibrosis: An Emerging Masquerader With a Sinister Presentation. Urology 2019; 133: 16-20 . doi:10.1016/j.urology.2019.06.007
    CrossrefPubMedGoogle Scholar
  • 6 Cristian S, Cristian M, Cristian P. et al Management of idiopathic retroperitoneal fibrosis from the urologist’s perspective. Ther Adv Urol 2015; 7: 85-99 . doi:10.1177/1756287214565637
    CrossrefPubMedGoogle Scholar
  • 7 Zhang S, Chen M, Li CM. et al Differentiation of Lymphoma Presenting as Retroperitoneal Mass and Retroperitoneal Fibrosis: Evaluation with Multidetector-row Computed Tomography. Chin Med J (Engl) 2017; 130: 691-697 . doi:10.4103/0366-6999.201606
    CrossrefPubMedGoogle Scholar
  • 8 Arora K, Rivera M, Ting DT. et al The histological diagnosis of IgG4-related disease on small biopsies: challenges and pitfalls. Histopathology 2019; 74: 688-698 . doi:10.1111/his.13787
    CrossrefPubMedGoogle Scholar
  • 9 Fernando A, Pattison J, Horsfield C. et al A lot of questions (and a few answers ...) in retroperitoneal fibrosis. BJU Int 2016; 117: 16-19 . doi:10.1111/bju.13061
    CrossrefPubMedGoogle Scholar
  • 10 Takahashi Y, Matsushima H, Mori T. et al Retroperitoneal fibrosis after chemo-radiotherapy for cervical cancer: A case report. J Obstet Gynaecol Res 2019; 45: 938-941 . doi:10.1111/jog.13898
    CrossrefPubMedGoogle Scholar
  • 11 Brandt AS, Dreger NM, Muller E. et al New (and old) aspects of retroperitoneal fibrosis. Urologe A 2017; 56: 887-894 . doi:10.1007/s00120-017-0428-y
    CrossrefPubMedGoogle Scholar
  • 12 Brandt AS, Kamper L, Kukuk S. et al Associated findings and complications of retroperitoneal fibrosis in 204 patients: results of a urological registry. J Urol 2011; 185: 526-531 . doi:10.1016/j.juro.2010.09.105
    CrossrefPubMedGoogle Scholar
  • 13 Ceresini G, Urban ML, Corradi D. et al Association between idiopathic retroperitoneal fibrosis and autoimmune thyroiditis: a case-control study. Autoimmun Rev 2015; 14: 16-22 . doi:10.1016/j.autrev.2014.08.006
    CrossrefPubMedGoogle Scholar
  • 14 Van Keerberghen CA, Harrouk A, Leone L. A new role for fluorine-18-fluorodeoxyglucose positron-emission tomography/computed tomography in Erdheim-Chester disease. World J Nucl Med 2019; 18: 201-203 . doi:10.4103/wjnm.WJNM_57_18
    Article in Thieme ConnectPubMedGoogle Scholar
  • 15 Knitza J, Kampylafka E, Wacker J. et al Erdheim-Chester disease: An important differential diagnosis and its main symptoms. Z Rheumatol 2019; 78: 66-71 . doi:10.1007/s00393-018-0566-7
    CrossrefPubMedGoogle Scholar
  • 16 Lian L, Wang C, Tian JL. IgG4-related retroperitoneal fibrosis: a newly characterized disease. Int J Rheum Dis 2016; 19: 1049-1055 . doi:10.1111/1756-185X.12863
    CrossrefPubMedGoogle Scholar
  • 17 Zen Y, Onodera M, Inoue D. et al Retroperitoneal fibrosis: a clinicopathologic study with respect to immunoglobulin G4. Am J Surg Pathol 2009; 33: 1833-1839 . doi:10.1097/pas.0b013e3181b72882
    CrossrefPubMedGoogle Scholar
  • 18 Mohammadzadeh Rezaei MA, Akhavan Rezayat A, Ardalan S. Retroperitoneal Fibrosis Due to Opium Abuse: A Case Series and Literature Review. Nephrourol Mon 2016; 8: e39788 . doi:10.5812/numonthly.39788
    CrossrefPubMedGoogle Scholar
  • 19 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: 181-188 . doi:10.7326/M13-2648
    CrossrefPubMedGoogle Scholar
  • 20 Moriconi D, Giannese D, Capecchi R. et al Risk factors for relapse and long-term outcome of idiopathic retroperitoneal fibrosis. Clin Exp Nephrol 2019; 23: 1147-1153 . doi:10.1007/s10157-019-01759-w
    CrossrefPubMedGoogle Scholar
  • 21 Kocot A, Riedmiller H. Treatment of long-term radiation injuries in the urinary tract. Urologe A 2015; 54: 1765-1771 . doi:10.1007/s00120-015-4011-0
    CrossrefPubMedGoogle Scholar
  • 22 Bertagna F, Treglia G, Leccisotti L. et al [(1)(8)F]FDG-PET/CT in patients affected by retroperitoneal fibrosis: a bicentric experience. Jpn J Radiol 2012; 30: 415-421 . doi:10.1007/s11604-012-0066-7
    CrossrefPubMedGoogle Scholar
  • 23 Gao L, Wang H, Xu Y. et al Computed tomography parameters can be used as predictive markers for the improvement of renal function in patients with retroperitoneal fibrosis. Clin Exp Rheumatol 2015; 33: 871-876
    PubMedGoogle Scholar
  • 24 Kamper L, Brandt AS, Ekamp H. et al The potential role of modern US in the follow-up of patients with retroperitoneal fibrosis. Diagn Interv Radiol 2014; 20: 3-8 . doi:10.5152/dir.2013.13132
    PubMedGoogle Scholar
  • 25 Kamper L, Brandt AS, Scharwachter C. et al MR evaluation of retroperitoneal fibrosis. Rofo 2011; 183: 721-726 . doi:10.1055/s-0031-1273450
    Article in Thieme ConnectPubMedGoogle Scholar
  • 26 Burn PR, Singh S, Barbar S. et al Role of gadolinium-enhanced magnetic resonance imaging in retroperitoneal fibrosis. Can Assoc Radiol J 2002; 53: 168-170
    PubMedGoogle Scholar
  • 27 Ruhlmann V, Poeppel TD, Brandt AS. et al (18)F-FDG PET/MRI evaluation of retroperitoneal fibrosis: a simultaneous multiparametric approach for diagnosing active disease. Eur J Nucl Med Mol Imaging 2016; 43: 1646-1652 . doi:10.1007/s00259-016-3351-3
    CrossrefPubMedGoogle Scholar
  • 28 Kamper L, Haage P, Brandt AS. et al Diffusion-weighted MRI in the follow-up of chronic periaortitis. Br J Radiol 2015; 88: 20150145 . doi:10.1259/bjr.20150145
    CrossrefPubMedGoogle Scholar
  • 29 Kamper L, Dreger NM, Brandt AS. et al Diffusion-weighted MRI and PET-CT in the follow up of chronic periaortitis. Int J Cardiovasc Imaging 2018; 34: 1779-1785 . doi:10.1007/s10554-018-1395-0
    CrossrefPubMedGoogle Scholar
  • 30 Bier G, Henes J, Eulenbruch C. et al Perfusion-based assessment of disease activity in untreated and treated patients with aortitis and chronic periaortitis: correlation with CT morphological, clinical and serological data. Br J Radiol 2015; 88: 20150526 . doi:10.1259/bjr.20150526
    CrossrefPubMedGoogle Scholar
  • 31 Bier G, Kurucay M, Henes J. et al Monitoring Disease Activity in Patients with Aortitis and Chronic Periaortitis Undergoing Immunosuppressive Therapy by Perfusion CT. Acad Radiol 2017; 24: 470-477 . doi:10.1016/j.acra.2016.10.013
    CrossrefPubMedGoogle Scholar
  • 32 Wiggermann P, Aust D, Stroszczynski C. et al Follow-up of FDG-PET/CT findings in retroperitoneal fibrosis associated with abdominal aortic aneurysm. Nuklearmedizin 2011; 50: N21-N22
    Article in Thieme ConnectPubMedGoogle Scholar
  • 33 Morin G, Mageau A, Benali K. et al Persistent FDG/PET CT uptake in idiopathic retroperitoneal fibrosis helps identifying patients at a higher risk for relapse. Eur J Intern Med 2019; 62: 67-71 . doi:10.1016/j.ejim.2019.01.019
    CrossrefPubMedGoogle Scholar
  • 34 Talati SJ, Abghari R, Kochkodan JJ. et al Use of Ga-67 imaging in diagnosis and follow-up after steroid treatment of retroperitoneal fibrosis. Clin Nucl Med 1995; 20: 995-997 . doi:10.1097/00003072-199511000-00012
    CrossrefPubMedGoogle Scholar
  • 35 Piccoli GB, Consiglio V, Arena V. et al Positron emission tomography as a tool for the ‘tailored’ management of retroperitoneal fibrosis: a nephro-urological experience. Nephrol Dial Transplant 2010; 25: 2603-2610 . doi:10.1093/ndt/gfq051
    CrossrefPubMedGoogle Scholar
  • 36 Koike Y, Matsui S, Takase K. et al CT-Guided Percutaneous Needle Biopsy in Patients with Suspected Retroperitoneal Fibrosis: A Retrospective Cohort Study. Cardiovasc Intervent Radiol 2019; 42: 1434-1440 . doi:10.1007/s00270-019-02266-x
    CrossrefPubMedGoogle Scholar
  • 37 Loricera J, Blanco R, Castaneda S. et al Tocilizumab in refractory aortitis: study on 16 patients and literature review. Clin Exp Rheumatol 2014; 32: S79-S89
    PubMedGoogle Scholar
  • 38 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) 226-227
    PubMedGoogle Scholar
  • 39 Dedinska I, Svetlik D, Adamicova K. et al Treatment of Ormond Disease and Idiopathic Membranous Glomerulonephritis by using Rituximab. Iran J Kidney Dis 2016; 10: 332-335
    PubMedGoogle Scholar
  • 40 Catanoso MG, Spaggiari L, Magnani L. et al Efficacy of infliximab in a patient with refractory idiopathic retroperitoneal fibrosis. Clin Exp Rheumatol 2012; 30: 776-778
    PubMedGoogle Scholar