Chronic Periaortitis (CP)
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.
KeywordsPositron Emission Tomography Compute Tomographic Angiography Magnetic Resonance Angiography Aortic Aneurysm Common Iliac Artery
- 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