Aortitis with dissection complicating systemic lupus erythematosus

Guard, Roger W.; Gotis-Graham, I; Edmonds, John; Thomas, Anthony

doi:10.1080/00313029500169023

Cited by 47 publications

(21 citation statements)

References 20 publications

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“…Acute aortic syndromes, including aortic dissection and rupture, can occur in persons with aortitis. 9,36,37 Inflammation-associated thrombus formation in the aortic lumen with peripheral embolization also has been reported. 38,39 The underlying cause of the aortitis and its concomitant manifestations determine the presenting symptoms.…”

Section: Clinical Presentationmentioning

confidence: 89%

Aortitis

2008

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Section: Clinical Presentationmentioning

confidence: 89%

Aortitis

2008

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“…Certain connective tissue diseases, e.g., rheumatoid arthritis, ankylosing spondylitis, systemic lupus erythematosus, and relapsing polychondritis, also occasionally lead to aortitis (3)(4)(5)(6)27). Sarcoidosis can cause aortitis and may be difficult to distinguish from Takayasu arteritis (7,28).…”

Section: Discussionmentioning

confidence: 99%

“…There are several major categories of inflammatory aortitis. First, aortitis is a complication of a number of primary systemic vasculitides or other rheumatologic conditions (2)(3)(4)(5)(6)(7). Diseases such as giant cell arteritis, Takayasu arteritis, rheumatoid arthritis, and related conditions usually cause lesions within the ascending aorta (8,9).…”

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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“…That study also showed thickened vessels in the adventitia similar to what we observed in our case. Aortitis has been described in case reports of SLE patients with aortic aneurysms, but it is not a common complication in SLE patients (6,15,16). A previous study reported increased numbers of lymphocytes and macrophages in ascending aortas with medial cystic degeneration, aneurysms, and dissection and implicated them in the observed increase in the numbers of apoptotic vascular smooth muscle cells (17).…”

Section: Discussionmentioning

confidence: 99%

Aneurysm of the ascending aorta in systemic lupus erythematosus: Case report and review of the literature

et al. 2017

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133Cardiovascular manifestations in patients with systemic lupus erythematosus (SLE) are common, but aortic aneurysm formation is rare. We present a 63-year-old male SLE patient with a two-year history of skin lesions, leucopenia, pericarditis, mitral valve vegetations consistent with Liebman-Sacks endocarditis, and an aneurysm of the ascending aorta, which was successfully repaired surgically. Histologic examination of the aneurysm showed medial cystic degeneration, smooth muscle necrosis, and mild adventitial perivascular lymphocytic aggregates. This histology is typical of thoracic aneurysms that carry a high risk for aortic dissection and patient death. The case highlights the importance of early detection and treatment of thoracic aortic aneurysms in patients with SLE.

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Aortitis with dissection complicating systemic lupus erythematosus

Cited by 47 publications

References 20 publications

Aortitis

Aortitis

IgG4‐related systemic disease and lymphoplasmacytic aortitis

Aneurysm of the ascending aorta in systemic lupus erythematosus: Case report and review of the literature

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