Idiopathic aortitis: an underrecognized vasculitis

Abstract

Aortitis is a general term denoting inflammation of the aortic wall. Various infectious and non-infectious diseases can be complicated by aortitis; in addition, isolated idiopathic aortitis has also been described. In a 12-year nationwide Danish population-based study, the prevalence of aortitis among 1,210 resected thoracic aorta samples was 6.1%, with nearly three-quarters of cases being idiopathic. Identified risk factors for aortitis included advanced age, a history of connective tissue disease, diabetes mellitus, and heart valve pathology. As in virtually all pathological studies, this study has a bias toward reporting the most severe cases of aortitis requiring surgical repair.

Inflammatory disease of the aorta: Patterns and classification of giant cell aortitis, Takayasu arteritis, and nonsyndromic aortitis

“…[1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20] To date, no universally accepted theory has been advanced that conclusively determines all forms of etiology, nor is there a generally accepted classification. Most authors would agree that there are those forms of aortitis that are clearly related to known bacterial or viral infections, such as syphilis, tuberculosis, human immunodeficiency disease, Kawasaki disease, and possibly lupus, in addition to frank mycotic aneurysms.…”

Comparison of idiopathic (isolated) aortitis and giant cell arteritis-related aortitis. A French retrospective multicenter study of 117 patients

“…The main underlying diseases causing non-infectious aortitis are giant cell arteritis (GCA) and Takayasu arteritis [2,3] and more rarely, sarcoidosis, Behçet's disease, Cogan syndrom, granulomatosis with polyangiitis, spondylarthropathy, IgG4-related disease, or relapsing polychondritis. In addition, idiopathic (isolated) aortitis (IA) has also been described [4]. Aortitis is related to significant morbidity and mortality through the development of aortic aneurysm, aortic wall rupture, aortic acute dissection, and/or thrombotic luminal obstruction [5].…”

Pulmonary oedema in the emergency room: what is hidden beyond an apparently common presentation

“…She also did not show any characteristic signs of TA: age of disease onset <40 year, claudication of the extremities, decreased brachial arterial pulse, blood pressure difference >10 mm Hg or audible bruit over the subclavian arteries or the aorta 12. There is an increasingly described and under-recognised vasculitis—idiopathic aortitis—virtually histologically indistinguishable from GCA and TA, which may be more common than previously thought 13. In a 12-year nationwide Danish population-based study, Schmidt et al 14 report a prevalence of 6.1% aortitis among 1.210 resected aorta surgical samples, with nearly three-quarters of cases being idiopathic; only 14% were linked to GCA or TA.…”

“…This disorder may be underdiagnosed for several reasons: the course of the disease may remain asymptomatic for a long time; patients are diagnosed when complications occur, mainly in the form of aortic aneurysms requiring surgery; nearly half of surgical samples are not submitted for pathological examination and some cases of idiopathic aortitis may not be recognised 14. It is therefore a condition deserving further epidemiological and pathophysiological studies, with emphasis in older female patients (mean age 73) and patients with diabetes, lying at the one end of a disease spectrum encompassing both GCA and TA 10 13. Three features are helpful to distinguish isolated idiopathic aortitis from other forms of ascending aortitis: absence of symptoms or signs of extra-aortic arteritis, absence of systemic disease such as autoimmune connective tissue disorders, and usually favourable outcome even in the absence of anti-inflammatory therapy 15 16.…”