Periaortitis: Gadolinium-enhanced Magnetic Resonance Imaging and Response to Therapy in Four Patients

Mitnick, Hal J.; Jacobowitz, Glenn R.; Krinsky, Glen; Eberle, Mark A.; Rosenzweig, Barry P.; Willis, David N; Rockman, Caron; Riles, Thomas S.

doi:10.1007/s10016-003-0060-8

Cited by 12 publications

(5 citation statements)

References 32 publications

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“…While periaortitis may be due to some of the systemic disorders listed above, chronic periaortitis can be present without clinical evidence of systemic vasculitis, particularly in the abdominal aorta [44][45][46]. This, again, likely represents a heterogeneous group of conditions and may be associated with a more generalized retroperitoneal fibrosis in some patients.…”

Section: Clinically Isolated Aortitismentioning

confidence: 91%

“…For cases on the other end of the spectrum, IAA has often been difficult to distinguish from chronic periaortitis due to outright systemic rheumatologic diseases including GCA, rheumatoid arthritis, ankylosing spondylitis (AS), systemic lupus erythematosus (SLE), GPA, and IgG4-RD [44][45][46]. Such cases of chronic periaortitis may also involve other arteries, present with clinical features of autoimmune diseases, and/or be accompanied by retroperitoneal fibrosis or sclerosing mediastinitis.…”

Section: Inflammatory Aortic (Atherosclerotic) Aneurysmsmentioning

confidence: 96%

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Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases

Stone¹,

Bruneval²,

Angelini³

et al. 2015

Cardiovascular Pathology

267

232

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Inflammatory diseases of the aorta include routine atherosclerosis, aortitis, periaortitis, and atherosclerosis with excessive inflammatory responses, such as inflammatory atherosclerotic aneurysms. The nomenclature and histologic features of these disorders are reviewed and discussed. In addition, diagnostic criteria are provided to distinguish between these disorders in surgical pathology specimens. An initial classification scheme is provided for aortitis and periaortitis based on the pattern of the inflammatory infiltrate: granulomatous/giant cell pattern, lymphoplasmacytic pattern, mixed inflammatory pattern, and the suppurative pattern. These inflammatory patterns are discussed in relation to specific systemic diseases including giant cell arteritis, Takayasu arteritis, granulomatosis with polyangiitis (Wegener's), rheumatoid arthritis, sarcoidosis, ankylosing spondylitis, Cogan syndrome, Behçet's disease, relapsing polychondritis, syphilitic aortitis, and bacterial and fungal infections.

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Section: Clinically Isolated Aortitismentioning

confidence: 91%

Section: Inflammatory Aortic (Atherosclerotic) Aneurysmsmentioning

confidence: 96%

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases

Stone¹,

Bruneval²,

Angelini³

et al. 2015

Cardiovascular Pathology

267

232

View full text Add to dashboard Cite

show abstract

“…The causes of chronic periaortitis have generally been viewed as distinct from the entities that cause thoracic aortitis. Chronic periaortitis encompasses idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysms, sometimes referred to together as perianeurysmal retroperitoneal fibrosis (4, 6, 7). In the medical literature, chronic periaortitis and perianeurysmal retroperitoneal fibrosis are often not delineated sufficiently from atherosclerosis of the abdominal aorta.…”

Section: Introductionmentioning

confidence: 99%

IgG4‐related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases

Stone¹,

Khosroshahi²,

Stone³

2010

Arthritis Care & Research

147

120

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Objective. IgG4-related systemic disease, a disorder recognized only recently, can cause lymphoplasmacytic inflammation in the thoracic aorta. The percentage of cases caused by IgG4-related systemic disease is not known. We aimed to determine the percentage of noninfectious thoracic aortitis cases that are associated with IgG4-related systemic disease and to establish pathologic criteria for identifying involvement of the thoracic aorta by this disorder. Methods. We searched our Pathology Service database to identify patients with noninfectious thoracic aortitis who underwent resection over a 5-year time span. The histologic features of these cases were reviewed. All cases of lymphoplasmacytic aortitis and representative cases of giant cell aortitis and atherosclerosis were stained by immunohistochemistry for IgG4 and for the plasma cell marker CD138. We determined the fraction of plasma cells that stained for IgG4. Results. Of 638 resected thoracic aortas, 33 (5.2%) contained noninfectious aortitis. Four of these cases (12% of all patients with noninfectious aortitis) had histologic features of lymphoplasmacytic aortitis. Three of those 4 cases (9% of the noninfectious aortitis cases) demonstrated pathologic involvement by

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“…Chronic periaortitis, in contrast to the other forms of idiopathic aortitis, involves the abdominal aorta (12). This designation encompasses idiopathic retroperitoneal fibrosis and inflammatory abdominal aortic aneurysms, sometimes referred to together as perianeurysmal retroperitoneal fibrosis (11,13,14). In general, overlap between chronic periaortitis and the other forms of idiopathic aortitis has been viewed as unusual.…”

mentioning

confidence: 99%

IgG4‐related systemic disease and lymphoplasmacytic aortitis

Stone¹,

Khosroshahi²,

Hilgenberg³

et al. 2009

Arthritis & Rheumatism

169

102

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We describe herein a patient who developed a dissection of the ascending aorta in the setting of IgG4-related systemic disease, linking IgG4-related systemic disease with a newly-recognized subset of noninfectious aortitis. At the time of aortic surgery, a transmural lymphoplasmacytic infiltrate was detected in the patient's aorta, with a principal focus of inflammation within the media. Immunohistochemical studies demonstrated that >50% of the plasma cells in the lesion stained for IgG4. By in situ hybridization, the plasma cells showed polytypic staining for kappa and lambda light chains, consistent with a polyclonal plasma cell infiltrate. Serologic evaluation revealed that the patient's IgG4 levels were elevated nearly 10-fold. Four years before aortic surgery, the patient had undergone a mediastinal lymph node biopsy. Reexamination of the lymph node revealed features consistent with IgG4-related systemic disease, which had not been recognized at the time of the original biopsy. Glucocorticoid therapy for the IgG4-related systemic disease yielded a prompt response. Recognition that IgG4-related systemic disease can involve the ascending as well as the descending abdominal aorta indicates the need for a change in the way idiopathic aortitis is regarded. This case offers new potential considerations for short-and long-term management of noninfectious aortitis, because of the frequent good response of IgG4-related systemic disease to glucocorticoid treatment without additional therapy. Treatment of the aortitis may prevent progression of the IgG4-related systemic disease to involvement of other organs. IgG4-related systemic disease should be considered in all patients with aortitis judged to be of unknown etiology.

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Periaortitis: Gadolinium-enhanced Magnetic Resonance Imaging and Response to Therapy in Four Patients

Cited by 12 publications

References 32 publications

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases

Consensus statement on surgical pathology of the aorta from the Society for Cardiovascular Pathology and the Association for European Cardiovascular Pathology: I. Inflammatory diseases

IgG4‐related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases

IgG4‐related systemic disease and lymphoplasmacytic aortitis

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