Skip to main content
SpringerLink
Log in

Chronische Periaortitis

Chronic periaortitis

  • Schwerpunkt
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Die chronische Periaortitis ist eine entzündlich-fibrosierende Erkrankung, die mit einer periaortalen Fibrosierung und Aortenaneurysmabildung einhergeht und meist retroperitoneal, gelegentlich auch thorakal lokalisiert ist. Eine entzündlich-vaskulitische Beteiligung großer Gefäße ist möglich. Neben Zeichen der systemischen Inflammation kommt es zu mechanischen Komplikationen, wobei die Obstruktion der Ureteren die häufigste ist. Die Diagnose basiert auf der kontrastmittelgestützten Computertomographie oder Magnetresonanztomographie, bei atypischen Befunden sollte sie bioptisch gesichert werden. Nach Ausschluss einer sekundären Genese, die eine Therapie der zugrunde liegenden Erkrankung notwendig machen würde, kann die idiopathische chronische Periaortitis mit Glukokortikoiden behandelt werden. Therapierefraktäre und zu Rezidiven neigende Verläufe können die Gabe weiterer immunsuppressiver Medikamente notwendig machen. Therapiedauer, Dosierung und Indikation der Zugabe immunsuppressiver Medikamente sind aktuell unklar; sie müssen durch weitere randomisierte, kontrollierte Studien mit größeren Fallzahlen definiert werden. Komplikationen können eine interventionelle oder operative Behandlung erfordern, bei Harnaufstau ist meist die Anlage von Doppel-J-Schienen ausreichend.

Abstract

Chronic periaortitis is an inflammatory and fibrosing disease presenting as periaortal fibrosis and formation of aortic aneurysms which are mostly localized in the retroperitoneum and occasionally in the mediastinum. Inflammatory vasculitic involvement of large vessels is also possible. In addition to symptoms of systemic inflammation, mechanical complications also occur whereby obstruction of the ureter is the most frequent. The diagnosis is made by contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) and if the findings are atypical the diagnosis should be confirmed by biopsy. After exclusion of a secondary genesis, in which case therapy of the underlying illness would be necessary, idiopathic chronic periaortitis can be treated with steroids. In cases of refractory and relapsing courses the administration of further immunosuppressive medication can be necessary. Duration of therapy, dosage and indications for immunosuppressive medication are currently unclear and have to be defined in further randomized controlled trials with larger cohorts. If complications occur, interventional or operative treatment can be necessary; in cases of hydronephrosis the placement of double-J-stents is usually sufficient.

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

Literatur

  1. Adler S, Lodermeyer S, Gaa J, Heemann U (2008) Successful mycophenolate mofetil therapy in nine patients with idiopathic retroperitoneal fibrosis. Rheumatology (Oxford) 47:1535–1538

    Google Scholar 

  2. Van Bommel EFH, Jansen I, Hendriksz TR, Aarnoudse ALHJ (2009) Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine 88:193–201

    Article  Google Scholar 

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

    Article  Google Scholar 

  4. Van Bommel EFH, Siemes C, Hak LE et al (2007) Long-term renal and patient outcome in idiopathic retroperitoneal fibrosis treated with prednisone. Am J Kidney Dis 49:615–625

    Article  Google Scholar 

  5. Brandt AS, Kamper L, Kukuk S et al (2013) An aid to decision-making in therapy of retroperitoneal fibrosis: dynamic enhancement analysis of gadolinium MRI. J Clin Med Res 5:49–56

    Google Scholar 

  6. Corradi D, Maestri R, Palmisano A et al (2007) Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int 72:742–753

    Article  CAS  PubMed  Google Scholar 

  7. Cronin CG, Lohan DG, Blake MA et al (2008) Retroperitoneal fibrosis: a review of clinical features and imaging findings. AJR Am J Roentgenol 191:423–431

    Article  PubMed  Google Scholar 

  8. Fry AC, Singh S, Gunda SS et al (2008) Successful use of steroids and ureteric stents in 24 patients with idiopathic retroperitoneal fibrosis: a retrospective study. Nephron Clin Pract 108:c213–c220

    Article  CAS  PubMed  Google Scholar 

  9. Higgins PM, Bennett-Jones DN, Naish PF, Aber GM (1988) Non-operative management of retroperitoneal fibrosis. Br J Surg 75:573–577

    Article  CAS  PubMed  Google Scholar 

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

    Google Scholar 

  11. Khosroshahi A, Carruthers MN, Stone JH et al (2013) Rethinking Ormond’s disease. Medicine 92:82–91

    Article  CAS  PubMed  Google Scholar 

  12. 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 

  13. Maritati F, Corradi D, Versari A et al (2012) Rituximab therapy for chronic periaortitis. Ann Rheum Dis 71:1262–1264

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

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

    Google Scholar 

  16. Salvarani C, Calamia KT, Matteson EL et al (2011) Vasculitis of the gastrointestinal tract in chronic periaortitis. Medicine 90:28–39

    Article  PubMed  Google Scholar 

  17. Salvarani C, Pipitone N, Versari A et al (2005) Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum 53:298–303

    Article  PubMed  Google Scholar 

  18. Scheel PJ Jr, Feeley N (2009) Retroperitoneal fibrosis: the clinical, laboratory, and radiographic presentation. Medicine 88:202–207

    Article  PubMed  Google Scholar 

  19. Scheel PJ Jr, Feeley N, Sozio SM (2011) Combined prednisone and mycophenolate mofetil treatment for retroperitoneal fibrosis: a case series. Ann Intern Med 154:31–36

    Article  PubMed  Google Scholar 

  20. Stone JR (2011) Aortitis, periaortitis, and retroperitoneal fibrosis, as manifestations of IgG4-related systemic disease. Curr Opin Rheumatol 23:88–94

    Article  CAS  PubMed  Google Scholar 

  21. Stone JH, Chan JKC, Deshpande V et al (2013) IgG4-related disease. Int J Rheumatol 2013:1–2

    Article  Google Scholar 

  22. Sultan S, Duffy S, Madhavan P et al (1999) Fifteen-year experience of transperitoneal management of inflammatory abdominal aortic aneurysms. Eur J Vasc Endovasc Surg 18:510–514

    Article  CAS  PubMed  Google Scholar 

  23. Swartz RD (2009) Idiopathic retroperitoneal fibrosis: a review of the pathogenesis and approaches to treatment. Am J Kidney Dis 54:546–553

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  25. Vaglio A, Palmisano A, Alberici F et al (2011) Prednisone versus tamoxifen in patients with idiopathic retroperitoneal fibrosis: an open-label randomised controlled trial. Lancet 378:338–346

    Article  CAS  PubMed  Google Scholar 

  26. Vaglio A, Pipitone N, Salvarani C (2011) Chronic periaortitis: a large-vessel vasculitis? Curr Opin Rheumatol 23:1–6

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  28. Vega J, Goecke H, Tapia H et al (2009) Treatment of idiopathic retroperitoneal fibrosis with colchicine and steroids: a case series. Am J Kidney Dis 53:628–637

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

Download references

Einhaltung ethischer Richtlinien

Interessenkonflikt. J.H. Schirmer, M. Both und F. Moosig geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

Author information

Authors and Affiliations

  1. Klinik für Rheumatologie und Immunologie, Klinikum Bad Bramstedt und Universitätsklinikum Schleswig-Holstein, Oskar-Alexander-Str. 26, 24576, Bad Bramstedt, Deutschland

    J.H. Schirmer & F. Moosig

  2. Klinik für Radiologie und Neuroradiologie, Universitätsklinikum Schleswig-Holstein, Campus Kiel, Kiel, Deutschland

    M. Both

Authors
  1. J.H. Schirmer
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Both
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. F. Moosig
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J.H. Schirmer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Schirmer, J., Both, M. & Moosig, F. Chronische Periaortitis. Internist 54, 1419–1426 (2013). https://doi.org/10.1007/s00108-013-3297-5

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-013-3297-5

Schlüsselwörter

Keywords

Search

Navigation