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Immunosuppressive treatment of chronic periaortitis: a retrospective study of 20 patients with chronic periaortitis and a review of the literature

K Warnatz¹, A G Keskin¹, M Uhl², C Scholz¹, A Katzenwadel³, P Vaith¹, H H Peter¹, U A Walker¹

¹ Department of Rheumatology and Clinical Immunology, Albert-Ludwigs University, Freiburg, Germany
² Department of Radiology, Albert-Ludwigs University, Freiburg, Germany
³ Department of Urology, Albert-Ludwigs University, Freiburg, Germany

Correspondence to:
Correspondence to:
Dr K Warnatz
Department of Rheumatology and Clinical Immunology, University Hospital Freiburg, Hugstetterstr 55, D-79106 Freiburg, Germany; warnatz{at}med1.ukl.uni-freiburg.de

Background: Retroperitoneal fibrosis (RPF) and inflammatory aneurysm of the abdominal aorta (IAAA) are regarded as two manifestations of the same disease, termed "chronic periaortitis".

Objective: To determine the optimal therapeutic and diagnostic approaches to IAAA.

Methods: The outcome of medical immunosuppressive and surgical treatment of 20 patients was examined. Measurements of the C reactive protein (CRP) were compared with contrast enhanced imaging studies in the follow up of the patients.

Results: The diameter of the periaortic mantle and its contrast enhancement improved in 13/15 (87%) patients given immunosuppressive treatment for a period of more than 6 months. Strong contrast enhancement was associated with a substantial rise in CRP, but no correlation between the CRP value and thickness of the fibrotic mass was found, even at intraindividual follow up.

Conclusions: Immunosuppressive treatment should be included in the first line treatment of patients with RPF and should be maintained long term. Imaging studies are better than CRP measurements in the evaluation of response to treatment.

Abbreviations: AZA, azathioprine; CRP, C reactive protein; CT, computed tomography; CYC, cyclophosphamide; DJ, double J; IAAA, inflammatory aneurysm of the abdominal aorta; MMF, mycophenolate mofetil; MRI, magnetic resonance imaging; OCS, oral corticosteroids; RPF, retroperitoneal fibrosis

Keywords: chronic periaortitis; Ormond’s disease; immunosuppression; aneurysms of the abdominal aorta; retroperitoneal fibrosis

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