Inflammatory diseases of the aorta

Abstract

Summary:Infl ammatory aortic diseases may occur with and without dilatation and are complicated by obstruction, rupture and dissection. Infections originate from periaortic foci or septicaemia and tend to result in the rapid development of aneurysms. Large vessel vasculitis due to Takayasu arteritis in younger and giant cell arteritis (GCA) in older patients is located in all layers of the aortic wall and prevails in the thoracic section. GCA patients are prone to developing aortic complications in the late course of disease. In Behçet's disease, aneurysms may have an unusual morphology and localisation. The diagnosis of aortitis is usually obtained by vascular imaging, but partly made only by biopsy on occasion of an operation, especially in case of isolated aortitis of the ascending aorta which mostly remains inapparent until dissection or large aneurysms have developed. Periaortitis typically occurs in the abdominal aorta and may lead to infl ammatory aortic aneurysm (IAA). It is looked upon as a special form of vasculitis, with an overlap to primary retroperitoneal fi brosis (RF). An identical pathology is discussed for the three diseases. On the other hand, about 50 % of isolated aortitides and periaortitides as well as retroperitoneal fi broses can be classed among IgG4-related diseases. Periaortitis also is observed after treatment of aortic aneurysms by stent-graft implantation. Special attention should be paid to ureteral obstruction along with RF or IAA. Once infection is ruled out, immunosuppression is applied to all forms of infl ammatory aortic diseases, primarily with glucocorticoids. However, after successful surgery for isolated thoracic aortitis or infl ammatory aortic aneurysm immunosuppression may be dispensable and it is not required if periaortic tissue enlargement persists in chronic inactive disease. For some patients with periaortitis and RF, tamoxifen may be a valuable alternative.

A case of recurrent acute aortic syndrome: Beyond emergency diagnosis and treatment

“…The definitive diagnosis is obtained with biopsy. [11] In our case, elevated levels of hsCRP and procalcitonin strengthened the hypothesis of infectious aetiology. Our patient had positive IgG Ab anti-Chlamydia Pneumoniae, which seems to be responsible of aortic wall degeneration through the production of metalloproteinases that destroy the matrix proteins of the aortic wall resulting in aneurysmal dilatation.…”

“…[10] Also medium-vessel vasculitis (polyarteritis nodosa and Kawasaki disease), ANCA-associated vasculitis and a new category called variable vessel vasculitis (Behcet's disease and Cogan's syndrome) can affect aorta, although seldom. [11] Furthermore, aortitis may be part of the spectrum of IgG4-related disease, an heterogeneous group of disorders, recently described, that can be responsible of 50% of isolated aortitis, periaortitis or retroperitoneal fibrosis. [11] In our patient IgG4 antibodies were normal.…”

“…[11] Furthermore, aortitis may be part of the spectrum of IgG4-related disease, an heterogeneous group of disorders, recently described, that can be responsible of 50% of isolated aortitis, periaortitis or retroperitoneal fibrosis. [11] In our patient IgG4 antibodies were normal.…”

“…[11] Also Treponema Pallidum can be responsible of aortic localizations almost entirely in the ascending aorta or in the aortic arch. [11] Negative VDRLtest made the diagnosis of syphilitic aortitis unlikely in our patient. Aortitis by Mycobacterium tuberculosis is a rare disease.…”

Aortic Aneurysm as a Complication of Granulomatosis with Polyangiitis Successfully Treated with Prednisolone and Cyclophosphamide: A Case Report and Review of the Literature

“…In our case, abdominal aneurysms showed findings of chronic periaortitis (CP) which were characterized by deposition of fibroinflammatory, periaortic tissue with the retroperitoneal tissue. The aortic diameter of CP is often normal at the time of diagnosis [ 5 ]. The differential diagnosis of CP includes abdominal aortic aneurysm, syphilitic mesoarteritis [ 6 ], IgG4-related disease (IgG4-RD) [ 7 ], Takayasu's arteritis [ 8 ], temporal arteritis [ 9 ], and infectious arteritis [ 10 ].…”