Skip to main content
SpringerLink
Log in

Retroperitoneale Fibrose

Retroperitoneal fibrosis

  • CME Weiterbildung • Zertifizierte Fortbildung
  • Published:
Der Urologe Aims and scope Submit manuscript

Zusammenfassung

Die retroperitoneale Fibrose (RPF) ist ein seltenes Krankheitsbild mit einem Erkrankungsgipfel in der 5. bis 7. Lebensdekade. Die Ätiologie ist weitgehend ungeklärt; mehrere Charakteristika der Erkrankung weisen jedoch auf ein autoimmunes Geschehen hin. Es wird zwischen der primären/idiopathischen und der sekundären RPF unterschieden. Die Symptomatik kann allgemein/unspezifisch oder lokalisiert sein. Zur Diagnosesicherung ist neben Anamnese, laborchemischen und radiologischen Untersuchungen häufig die Entnahme von Gewebe aus den entsprechenden Arealen erforderlich. Die Therapie sollte zunächst die Funktion der betroffenen (Hohl-) Organe wiederherstellen. Anschließend wird eine medikamentöse Therapie zum Überführen des akut fortschreitenden Stadiums in eine stabile Phase der Erkrankung eingeleitet. Bei einem Teil der Patienten wird allerdings eine definitive Harnleiterchirurgie im weiteren Verlauf erforderlich. Da es sich bei der RPF um ein potenziell chronisch rezidivierendes Krankheitsbild handelt, ist eine lebenslange Nachsorge der Patienten erforderlich.

Abstract

Retroperitoneal Fibrosis (RPF) is a rare disease with peak incidence in the fifth to seventh decades of life. Its etiology remains to a great extent unclear; however, many characteristics of the disease suggest an autoimmune origin. Distinction is made between primary/idiopathic and secondary RPF. Symptoms may be general/nonspecific or localized (due to replacement or compression of organs). In addition to patient history, laboratory analysis and radiologic examinations, diagnosis often requires biopsy taken from the area affected. After restoring the function of involved (hollow) organs, medical therapy with prednisone, immunosuppressive drugs or tamoxifen is aimed at converting active disease to stable disease. However, some patients require ureteral surgery due to unsuccessful treatment. Life-long follow-up of patients is necessary since RPF can show a chronically relapsing course.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4

Literatur

  1. Abercrombie GF, Vinnicombe J (1980) Retroperitoneal fibrosis. Practical problems and management. Br J Urol 52: 443–445

    PubMed  CAS  Google Scholar 

  2. Afeltra A, Genilucci UV, Rabitti C et al. (2005) Retroperitoneal fibrosis and ankylosing spondylitis: which links? Semin Arthritis Rheum 35: 43–48

    Article  PubMed  Google Scholar 

  3. Albarran J (1905) Retention renale par peri-ureterie: liberation externe de l’uretere. Assoc Fr Urol 9: 511–517

    Google Scholar 

  4. Al-Musawi D, Mitchenere P, Al-Akraa M (1998) Idiopathic retroperitoneal fibrosis treated with tamoxifen only. Br J Urol 82: 442–443

    PubMed  CAS  Google Scholar 

  5. Amis ES Jr (1991) Retroperitoneal fibrosis. AJR Am J Roentgenol 157: 321–329

    PubMed  Google Scholar 

  6. Baker LRI, Mallinson WJW, Gregory MC et al. (1988) Idiopathic retroperitoneal fibrosis. A retrospective analysis of 60 cases. Br J Urol 60: 497–503

    Google Scholar 

  7. Benedict MF, Niiesch R, Gasser TC (1993) Retroperitoneal fibrosis and chronic periaortitis: a new hypothesis. Eur Urol 23: 371–374

    Google Scholar 

  8. van Bommel EFH, Hendriksz TR, Huiskes AWLC, Zeegers AGM (2006) Brief communication: tamoxifen therapy for nonmalignant retroperitoneal fibrosis. Ann Intern Med 144: 101–106

    Google Scholar 

  9. Buff DD, Bogin MB, Faltz LL (1989) Retroperitoneal fibrosis. A report of selected cases and a review of the literature. N Y State J Med 89: 511–516

    PubMed  CAS  Google Scholar 

  10. Burn PR, Singh S, Barbar S et al. (2002) Role of gadolinium-enhanced magnetic resonance imaging in retroperitoneal fibrosis. Can Assoc Radiol J 53: 168–170

    PubMed  Google Scholar 

  11. Chizzolini C, Raschi E, Rezzonico R et al. (2002) Autoantibodies to fibroblasts induce a proadhesive and proinflammatory fibroblast phenotype in patients with systemic sclerosis. Arthritis Rheumatol 46: 1602–1613

    Article  CAS  Google Scholar 

  12. Clark CP, Vanderpool D, Preskitt JT (1991) The response of retroperitoneal fibrosis to tamoxifen. Surgery 109: 502–506

    PubMed  CAS  Google Scholar 

  13. Desgrandchamps F, Paulhac P, Fornairon S et al. (1998) Artificial ureteral replacement for ureteral necrosis after renal transplantation: report of 3 cases. J Urol 159: 1830–1832

    Article  PubMed  CAS  Google Scholar 

  14. Drieskens O, Blockmans D, van den Bruel A, Mortelmans L (2002) Riedel’s thyroiditis and retorperitoneal fibrosis in multifocal fibrosclerosis: positron emission tomography findings. Clin Nucl Med 27: 413–415

    Article  PubMed  Google Scholar 

  15. Duvic C, Desrame J, Leveque C, Nedelec G (2004) Retroperitoneal fibrosis, sclerosing pancreatitis and bronchiolitis obliterans with organizing pneumonia. Nephrol Dial Transplant 19: 2397–2399

    Article  PubMed  CAS  Google Scholar 

  16. Elashry OM, Nakada SY, Wolf JS Jr et al. (1996) Ureterolysis for extrinsic ureteral obstruction: a comparison of laparoscopic and open surgical techniques. J Urol 156: 1403–1410

    Article  PubMed  CAS  Google Scholar 

  17. Engelhardt PF, Riedl CR, Plas E et al. (2000) Pergolid-Mesylat (Permax-TM) induzierte sekundäre retroperitoneale Fibrose. J Urol Urogynäkol 5: 48–50

    Google Scholar 

  18. Fowler JW (1987) Peritoneal flap ureteropexy for idiopathic retroperitoneal fibrosis. Br J Urol 60: 18–22

    PubMed  CAS  Google Scholar 

  19. García Peñalver C, Tejido Sánchez A, Suárez Charneco A et al. (2002) Surgery for idiopathic retroperitoneal fibrosis by ureterolysis and reteric protection with a posterior pre-peritoneal fat flap. BJU International 89: 783–786

    Article  Google Scholar 

  20. Gilkeson GS, Allen NB (1996) Retroperitoneal fibrosis: a true connective tissue disease. Rheum Dis Clin North Am 22: 23–38

    Article  PubMed  CAS  Google Scholar 

  21. Grotz W, von Zedtwitz I, Andre M, Schollmeyer P (1998) Treatment of retroperitoneal fibrosis by mycophenolate mofetil and corticosteroids. Lancet 352: 1195

    Article  PubMed  CAS  Google Scholar 

  22. Hauck EW, Hauptmann A, Weidner W et al. (2003) Prospective analysis of HLA classes I and II antigen frequency in patients with peyronie’s disease. J Urol 170: 1443–1446

    Article  PubMed  CAS  Google Scholar 

  23. Heidenreich A, Derakhshani P, Neubauer S, Krug B (2000) Behandlungsergebnisse der primären und sekundären retroperitonealen Fibrose. Urologe 39: 141–148

    Article  PubMed  CAS  Google Scholar 

  24. von Heyden B, Roth S, Hertle L (1996) Medikamentöse Therapie der retroperitonealen Fibrose. Aktuelle Urologie 27: 6–11

    Google Scholar 

  25. Hamano H, Kawa S, Ochi Y et al. (2002) Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet 359: 1403–1404

    Article  PubMed  Google Scholar 

  26. Higgins PM, Aber GM (1990) Idiopathic retroperitoneal fibrosis: an update. Dig Dis 8: 206–222

    PubMed  CAS  Google Scholar 

  27. Jabbour ME, Desgrandchamps F, Angelescu E et al. (2001) Percutaneous implantation of subcutaneous prosthetic ureters: long term outcome. J Endourol 15: 611–614

    Article  PubMed  CAS  Google Scholar 

  28. Kardar AH, Kattan S, Lindstedt E, Hanash K (2002) Steroid therapy for idiopathic retroperitoneal fibrosis: dose and duration. J Urol 168: 550–555

    Article  PubMed  CAS  Google Scholar 

  29. Kerr WS, Suby HI, Vickery A, Fraley F (1968) Idiopathic retroperitoneal fibrosis-clinical experience with 15 cases. J Urol 99: 575–584

    PubMed  Google Scholar 

  30. Kinder CH (1972) Retroperitoneal fibrosis. J R Soc Med 72: 485–486

    Google Scholar 

  31. Koep L, Zuidema GD (1977) The clinical significance of retroperitoneal fibrosis. Surgery 81: 250

    PubMed  CAS  Google Scholar 

  32. Kottra JJ, Dunnick NR (1996) Retroperitoneal fibrosis. Radiol Clin North Am 43: 44–49

    Google Scholar 

  33. Kunkler RB, Osborn DE, Abbott RJ (1998) Retorperitoneal fibrosis caused by treatment with pergolide in a patient with Parkinson’s disease. BJU Int 82: 147

    Article  CAS  Google Scholar 

  34. Lepor H, Walsh P (1979) Idiopathic retroperitoneal fibrosis. J Urol 122: 1–6

    PubMed  CAS  Google Scholar 

  35. Loffeld RJLF, van Weel THF (1993) Tamoxifen for retroperitoneal fibrosis. Lancet 341: 382

    Article  PubMed  CAS  Google Scholar 

  36. Marcolongo R, Tavolini IM, Laveder F et al. (2004) Immunosuppressive therapy for idiopathic retroperitoneal fibrosis: a retrospective analysis of 26 cases. Am J Med 116: 194–197

    Article  PubMed  Google Scholar 

  37. Martorana D, Vagio A, Greco P et al. (2006) Chronic periaortitis and HLA-DRBI*03: another due to an autoimmune origin. Arthritis Rheum 55: 126–130

    Article  PubMed  CAS  Google Scholar 

  38. Marzano A, Tranai A, Leone N et al. (2001) Treatment of idiopathic retroperitoneal fibrosis using cyclosporin. Ann Rheum Dis 60: 427–428

    Article  PubMed  CAS  Google Scholar 

  39. Meschi M, Truss MC, Schultheiss D et al. (1998) Long-term follow-up of patients with reptroperitoneal fibrosis. J Urol 161: 75

    Google Scholar 

  40. Miller OF, Smith LJ, Ferrara EX et al. (2003) Presentation of idiopathic retroperitoneal fibrosis in the pediatric population. J Pediatr Surg 38: 1685–1688

    Article  PubMed  Google Scholar 

  41. Mitchinson MJ (1970) The pathology of idiopathic retroperitoneal fibrosis. J Clin Pathol 23: 681–689

    Article  PubMed  CAS  Google Scholar 

  42. Mitchinson MJ (1972) Aortic disease in idiopathic retroperitoneal and mediastinal fibrosis. J Clin Pathol 25: 287–293

    Article  PubMed  CAS  Google Scholar 

  43. Mitchinson MJ (1984) Chronic periaortitis and periarteritis. Histopathology 8: 589–600

    Article  PubMed  CAS  Google Scholar 

  44. Mohr MTJ, Altwein JE (1992) Retroperitoneale Fibrose. Therapie urologischer Erkrankungen. Enke, Stuttgart

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

    Article  PubMed  CAS  Google Scholar 

  46. Nitz GL, Hewitt CB, Straffon RA et al. (1970) Retroperitoneal malignancy masquerading as bening retroperitoneal fibrosis. J Urol 103: 46

    PubMed  CAS  Google Scholar 

  47. Parums DV (1990) The spectrum of chronic periaortitis. Histopathology 16: 423–431

    Article  PubMed  CAS  Google Scholar 

  48. Parums DV, Brown DL, Mitchinson MJ (1990) Serum antibodies to oxidized low-densitiy lipoproteins and ceroid in chronic periaortitis. Arch Pathol Lab Med 114: 383–387

    PubMed  CAS  Google Scholar 

  49. Poitras JM, Dennis GJ, Reed W (2000) Clinical images: diagnosis and treatment of retroperitoneal fibrosis (Ormond’s disease). Arthritis Rheumatol 43: 943

    CAS  Google Scholar 

  50. Reiher F, Steinbach F, Lippert H et al. (1998) Ausgedehnte multifokale retro- und intraperitoneale Fibrose (Morbus Ormond). Aktuelle Urologie 29: 361–363

    Article  Google Scholar 

  51. Ronda N, Gatti R, Giacosa R et al. (2002) Antifibroblast antibodies from systemic sclerosis patients are internalized by fibroblasts via a caveolin-linked pathway. Arthritis Rheumatol 46: 1595–1601

    Article  CAS  Google Scholar 

  52. Salliot C, Lavie F, Azria A et al. (2005) Retroperitoneal fibrosis secondary to spondylodiscitis after infection with prevotella. J Rheumatol 32: 957–958

    PubMed  Google Scholar 

  53. Saxton HM, Kilpatrick FR, Kinder CH et al. (1969) Retroperitoneal fibrosis. A radiological and follow-up study of fourteen cases. QJM 38: 159

    PubMed  CAS  Google Scholar 

  54. Shokeir AA, El-Diasty T, Eassa W et al. (2004) Diagnosis of ureteral obstruction in patients with compromised renal function: the role of non-invasive imaging modalities. J Urol 171: 2303–2306

    Article  PubMed  Google Scholar 

  55. Sigel A, Wach M (1994) Die primäre retroperitoneal Fibrose in erweiterter Sicht. Klinikarzt 9: 367–372

    Google Scholar 

  56. Streem SB et al. Chapter 13, Campbell’s urology, 8th edn. Saunders, New York, pp 505–508

  57. Thiele A, Störkel S, Stierle HE (1998) Retroperitonealfibrose und Arthritis – Manifestation derselben Erkrankung. Z Rheumatol 57: 285–291

    Article  PubMed  CAS  Google Scholar 

  58. Tresidder GD, Blandy JP, Singh M (1972) Omental sleeve to prevent retroperitoneal fibrosis around the ureter. Urol Intern 27: 144–148

    CAS  Google Scholar 

  59. Ubrig B, Waldner M, Roth S (2001) Reconstruction of ureter with transverse retubularized colon segments. J Urol 166: 973–976

    Article  PubMed  CAS  Google Scholar 

  60. Uibu T, Oksa P, Auvinen A et al. (2004) Asbestos exposures as a risk factor for retroperitoneal fibrosis. Lancet 363: 1422–1426

    Article  PubMed  CAS  Google Scholar 

  61. Vaglio A, Corradi D, Manenti L et al. (2003) Evidence of autoimmunitiy in chronic periaortitis: a prospective study. Am J Med 114: 454–462

    Article  PubMed  Google Scholar 

  62. Vaglio A, Buzio C (2005) Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol 17: 34–40

    Article  PubMed  Google Scholar 

  63. Vaglio A, Salvarani C, Buzio C (2006) Retroperitoneal fibrosis. Lancet 367: 241–251

    Article  PubMed  Google Scholar 

  64. Vivas I, Nicloas AI, Velazquez P et al. (2000) Retroperitoneal fibrosis: typical and atypical manifestations. Br J Radiol 73: 214–222

    PubMed  CAS  Google Scholar 

  65. Wagenknecht LV, Hardy JC (1978) Retroperitoneale Fibrosen. Thieme, Stuttgart

  66. Wagenknecht LV, Hardy JC (1981) Value of various treatments for retroperitoneal fibrosis. Eur Urol 7: 193–200

    PubMed  CAS  Google Scholar 

  67. Waldner M, Hertle L, Roth S (1999) Ileal ureteral substitution in reconstructive urological surgery: is an antireflux procedure necessary? J Urol 162: 323–326

    Article  PubMed  CAS  Google Scholar 

  68. Warnatz K, Keskin AG, Uhl M et al. (2005) Immunosuppressive treatment of chronic periaortitis: a retrospective study of 20 patients with chronic periaottitis and a review of the literature. Ann Rheum Dis 64: 828–833

    Article  PubMed  CAS  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Klinik für Urologie und Kinderurologie, HELIOS Klinikum Wuppertal, Universitätsklinikum der Universität Witten/Herdecke, Heusnerstraße 40, 42283, Wuppertal, Deutschland

    S. Burkhardt Soares, S. Kukuk, A.S. Brandt, A. Fehr & S. Roth

Authors
  1. S. Burkhardt Soares
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. S. Kukuk
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A.S. Brandt
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. Fehr
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Roth
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to S. Burkhardt Soares.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Burkhardt Soares, S., Kukuk, S., Brandt, A. et al. Retroperitoneale Fibrose. Urologe 47, 489–500 (2008). https://doi.org/10.1007/s00120-008-1705-6

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00120-008-1705-6

Schlüsselwörter

Keywords

Search

Navigation