Chronic Periaortitis (CP)

  • Nicolò Pipitone
  • Augusto Vaglio
  • Carlo Salvarani
  • Anthony W. Stanson
Part of the Medical Radiology book series (MEDRAD)


Chronic periaortitis is a rare, but treatable immune-mediated inflammatory disorder affecting the infrarenal abdominal adventitia of the aorta. The inflammation is a major etiology causing retroperitoneal fibrosis that can lead to entrapment of the ureters and rarely obliterate the lumen of the inferior vena cava. Patient symptoms include: back pain, abdominal pain and tenderness, malaise, and fever. Infrarenal aortic aneurysms are associated in a minority of patients. In the majority of cases, inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) are elevated as are white blood cells; urea nitrogen and creatinine may be elevated in the presence of urinary flow obstruction. Rarely, variants of periaortitis may involve the aorta above the renal artery, including extension to the thoracic aorta. These variant forms may additionally result in stenosis of the aorta, iliac arteries and other primary branches of the aorta. Imaging techniques, mainly computerized tomography and magnetic resonance imaging, have a crucial role in establishing the diagnosis and detecting an aortic aneurysm. PET scans may confirm active disease as well as identify widespread large artery vasculitis. Glucocorticoids are the cornerstone of therapy, but immunosuppressive agents should be used in relapsing cases.


Positron Emission Tomography Compute Tomographic Angiography Magnetic Resonance Angiography Aortic Aneurysm Common Iliac Artery 
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  1. Andersohn F, Garbe E (2009) Cardiac and noncardiac fibrotic reactions caused by ergot-and nonergot-derived dopamine agonists. Mov Disord 24(1):129–133PubMedCrossRefGoogle Scholar
  2. Bilici A, Karadag B, Doventas A et al (2004) Retroperitoneal fibrosis caused by pergolide in a patient with Parkinson’s disease. Neth J Med 62(10):389–392PubMedGoogle Scholar
  3. Boiardi L, Vaglio A, Nicoli D, Farnetti E, Palmisano A, Pipitone N, Maritati F, Casali B, Martorana D, Moroni G, Gallelli B, Buzio C, Salvarani C. CC chemokine receptor 5 polymorphism in chronic periaortitis. Rheumatology (Oxford). 2011 Jan 21. [Epub ahead of print]Google Scholar
  4. Chander S, Ergun EL, Chugani HT et al (2002) High 2-deoxy-2-[18F]fluoro-d-glucose accumulation in a case of retroperitoneal fibrosis following resection of carcinoid tumor. Mol Imaging Biol 4(5):363–368PubMedCrossRefGoogle Scholar
  5. Coppi G, Rametta F, Aiello S et al (2010) Inflammatory abdominal aortic aneurysm endovascular repair into the long-term follow-up. Ann Vasc Surg 24(8):1053–1059PubMedCrossRefGoogle Scholar
  6. Corradi D, Maestri R, Palmisano A et al (2007) Idiopathic retroperitoneal fibrosis: clinicopathologic features and differential diagnosis. Kidney Int 72(6):742–753PubMedCrossRefGoogle Scholar
  7. Kottra JJ, Dunnick NR (1996) Retroperitoneal fibrosis. Radiol Clin North Am 34(6):1259–1275PubMedGoogle Scholar
  8. Martorana D, Vaglio A, Greco P et al (2006) Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin. Arthritis Rheum 55(1):126–130PubMedCrossRefGoogle Scholar
  9. Modlin IM, Shapiro MD, Kidd M (2004) Carcinoid tumors and fibrosis: an association with no explanation. Am J Gastroenterol 99(12):2466–2478PubMedCrossRefGoogle Scholar
  10. Nitecki SS, Hallett JW Jr, Stanson AW et al (1996) Inflammatory abdominal aortic aneurysms: a case-control study. J Vasc Surg 23(5):860–868 discussion 868–869PubMedCrossRefGoogle Scholar
  11. Palmisano A, Vaglio A (2009) Chronic periaortitis: a fibro-inflammatory disorder. Best Pract Res Clin Rheumatol 23(3):339–353PubMedCrossRefGoogle Scholar
  12. Parums DV, Brown DL, Mitchinson MJ (1990) Serum antibodies to oxidized low-density lipoprotein and ceroid in chronic periaortitis. Arch Pathol Lab Med 114(4):383–387PubMedGoogle Scholar
  13. Pipitone N, Versari A, Salvarani C (2008) Role of imaging studies in the diagnosis and follow-up of large-vessel vasculitis: an update. Rheumatology (Oxford) 47(4):403–408CrossRefGoogle Scholar
  14. Ramshaw AL, Roskell DE, Parums DV (1994) Cytokine gene expression in aortic adventitial inflammation associated with advanced atherosclerosis (chronic periaortitis). J Clin Pathol 47(8):721–727PubMedCrossRefGoogle Scholar
  15. Salvarani C, Pipitone N, Versari A et al (2005) Positron emission tomography (PET): evaluation of chronic periaortitis. Arthritis Rheum 53(2):298–303PubMedCrossRefGoogle Scholar
  16. Teruya TH, Abou-Zamzam AM Jr, Ballard JL (2001) Inflammatory abdominal aortic aneurysm treated by endovascular stent grafting: a case report. Vasc Surg 35(5):391–395PubMedGoogle Scholar
  17. Uibu T, Oksa P, Auvinen A et al (2004) Asbestos exposure as a risk factor for retroperitoneal fibrosis. Lancet 363(9419):1422–1426PubMedCrossRefGoogle Scholar
  18. Vaglio A, Buzio C (2005) Chronic periaortitis: a spectrum of diseases. Curr Opin Rheumatol 17(1):34–40PubMedCrossRefGoogle Scholar
  19. Vaglio A, Corradi D, Manenti L et al (2003) Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 114(6):454–462PubMedCrossRefGoogle Scholar
  20. Vaglio A, Greco P, Versari A et al (2005) 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 23(2):231–234PubMedGoogle Scholar
  21. Vaglio A, Salvarani C, Buzio C (2006) Retroperitoneal fibrosis. Lancet 367(9506):241–251PubMedCrossRefGoogle Scholar
  22. Vaglio A, Palmisano A, Corradi D et al (2007) Retroperitoneal fibrosis: evolving concepts. Rheum Dis Clin North Am 33(4):803–817 vi–viiPubMedCrossRefGoogle Scholar
  23. van Bommel EF, 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(5):615–625PubMedCrossRefGoogle Scholar
  24. van Bommel EF, van der Veer SJ, Hendriksz TR et al (2008) Persistent chronic peri-aortitis (‘inflammatory aneurysm’) after abdominal aortic aneurysm repair: systematic review of the literature. Vasc Med 13(4):293–303PubMedCrossRefGoogle Scholar
  25. van Bommel EF, Jansen I, Hendriksz TR et al (2009) Idiopathic retroperitoneal fibrosis: prospective evaluation of incidence and clinicoradiologic presentation. Medicine (Baltimore) 88(4):193–201CrossRefGoogle Scholar
  26. Walter MA, Melzer RA, Schindler C, Muller-Brand J, Tyndall A, Nitzsche EU (2005) The value of (18F) FDG-PET in the diagnosis of large-vessel vasculitis and the assessment of activity and extent of disease. Eur J Nucl Med Mol Imaging 32:674–681PubMedCrossRefGoogle Scholar
  27. 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(6):828–833PubMedCrossRefGoogle Scholar
  28. Young PM, Peterson JJ, Calamia KT (2008) Hypermetabolic activity in patients with active retroperitoneal fibrosis on F-18 FDG PET: report of three cases. Ann Nucl Med 22(1):87–92PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2011

Authors and Affiliations

  • Nicolò Pipitone
    • 1
  • Augusto Vaglio
    • 2
  • Carlo Salvarani
    • 1
  • Anthony W. Stanson
    • 3
  1. 1.Divisione di ReumatologiaArcispedale Santa Maria NuovaReggio EmiliaItaly
  2. 2.Dipartimento di Clinica Medica, Nefrologia e Scienze della PrevenzioneUniversità degli Studi di ParmaParmaItaly
  3. 3.Department of RadiologyMayo Clinic College of MedicineRochesterUSA

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