Aortitis with aneurysm formation as a rare complication of Wegener's granulomatosis

Ünlü, Çağdaş; Willems, M.C.; Berge, Ineke J. M. ten; Legemate, Dink A.

doi:10.1016/j.jvs.2011.05.014

Cited by 21 publications

(11 citation statements)

References 9 publications

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“…In rare cases, ANCA-associated vasculitides [45] and aneurysm-forming polyangitis nodosa may include the aorta. It can also be involved in autoimmune disorders.…”

Section: Other Vasculitides and Rheumatologic Disordersmentioning

confidence: 99%

Inflammatory diseases of the aorta

Caspary

2016

Vasa

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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.

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“…In rare cases, ANCA-associated vasculitides [45] and aneurysm-forming polyangitis nodosa may include the aorta. It can also be involved in autoimmune disorders.…”

Section: Other Vasculitides and Rheumatologic Disordersmentioning

confidence: 99%

Inflammatory diseases of the aorta

Caspary

2016

Vasa

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“…Histology is only available for the four cases in which surgical treatment was required, and biopsies of the aortic tissue revealed vasculitis suggestive of GPA. Of the remaining two patients, one died due to aortic dissection and one received medical treatment [ [12] , [13] , [14] , [15] , [16] .…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal fibrosis with periaortitis: A case report of an unusual form of presentation of granulomatosis with polyangiitis

Revilla

Fernández

Ramírez

et al. 2016

Respiratory Medicine Case Reports

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Granulomatosis with polyangiitis (GPA) is the name that has been used in recent years for Wegener's granulomatosis. This condition is a systemic inflammatory disease characterised by necrotizing vasculitis that affects small and medium-sized blood vessels (capillaries, arterioles, venules and arteries). The granulomatous inflammation affects the respiratory system; it also commonly affects the kidney and can very rarely affect large vessels such as the aorta and the surrounding retroperitoneal tissue. Early diagnosis and treatment is of vital importance because of the high risk of dissection and of obstruction of retroperitoneal structures.We present the case of a 74-year-old man with a past history of infrarenal abdominal aortic aneurysm. He consulted for abdominal pain. Cavitating pulmonary nodules and retroperitoneal fibrosis with periaortic alterations were detected on computed tomography. Laboratory investigations revealed that the patient was positive for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and necrotizing granulomas were observed on biopsies of the lung lesions and retroperitoneal tissue. The patient was diagnosed with GPA and treatment was started with glucocorticoids and immunosuppressive agents, which led to a significant clinical and radiological improvement over the following months.

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“…En los casos publicados de asociación de vasculitis en vasos pequeños y grandes, la ocurrencia ha sido simultánea en la mayoría de los casos. Cuando fue metacrónica, el intervalo varió entre 6 meses y 22 años [5][6][7]9,14 . En esta paciente fue 9 años.…”

Section: Discussionunclassified

“…Otros autores han planteado que una vasculitis de las arterias retroperitoneales (periaortitis) invadiría la pared de la aorta 3,7 . Las biopsias efectuadas durante la reparación de aneurismas, revascularización o necropsias han revelado vasculitis de vasa vasorum y arterias retroperitoneales, inflamación, granulomas, rotura de las fibras elásticas de la media, necrosis fibrinoide y vasculitis necrotizante granulomatosa [2][3][4][5][6][7][8][9]19 . Si bien estos hallazgos son comunes con las vasculitis de grandes vasos clásicas, la necrosis parece ser un hecho distintivo las vasculitis ANCA(+) 3 .…”

Section: Discussionunclassified