Chronic Periaortitis – Review and Presentations

ABSTRACT

Chronic periaortitis is a rare inflammatory condition predominantly affecting the abdominal segment of the aorta. This can present as IgG4 related inflammatory disease, idiopathic retroperitoneal fibrosis, perianeurysmal retroperitoneal fibrosis andinflammatory abdominal aortic aneurysm (IAAA). Aortitis can also be a manifestation of a number of rheumatological large vessel vasculitides such as Takayasu arteritis and giant cell arteritis (GCA). We present three interesting cases of chronic periaortitis and a literature review. The first case showsa classic picture ofIgG4 periaortitis. The second case illustrates periaortitis with retroperitoneal fibrosis, ureteric involvement and hydronephrosis, following abdominal aortic aneurysmal stenting. The final case presents as widespread periaortitis due to Takayasu's disease involving the entire aorta including the arch and root of the subclavian artery