Behçet's aortitis and aortic regurgitation: a report of two cases

Tsui, Kin-Lam; Lee, Ka-Wing; Chan, Wen‐Hsiung; Chan, Helen Ka-Yan; Hon, Sonny F K; Leung, Tat-Chi; Lee, Ka-Lai; Tsoi, Tak Hong; Li, Shu-Kin

doi:10.1016/j.echo.2003.09.009

Cited by 26 publications

(9 citation statements)

References 9 publications

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“…9 The thoracic aorta and pulmonary arteries had the most severe lesions, accounting for 40% of deaths. Aortitis is infrequent in BD 29,31 and was found in only 3% of patients in the current study. Arterial aneurysms are usually considered the most severe lesions because they expose the patient to the risk of rupture.…”

Section: Discussioncontrasting

confidence: 56%

Long-Term Outcome of Arterial Lesions in Behçet Disease

et al. 2012

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The vasculitis of Behçet disease (BD) is distinctive because of involvement of both arteries and veins of all sizes. The concept of vasculo-Behçet disease has been adopted for cases in which vascular manifestations are present and often dominate the clinical features. While venous manifestations are frequent and have been reported in many publications, data regarding arterial lesions in patients with BD are rare and often isolated. In this study, we report the main characteristics, treatment, and long-term outcome of 101 patients with arterial lesions among a cohort of 820 (12.3%) BD patients. Factors that affect prognosis were assessed by multivariate analysis. There were 93 (91.2%) male patients; the median (Q1-Q3) age at diagnosis of BD was 33 (27-41) years. Arterial lesions included aneurysms (47.3%), occlusions (36.5%), stenosis (13.5%), and aortitis (2.7%). Lesions mainly involved the aorta (n = 25) and femoral (n = 23) and pulmonary (n = 21) arteries. Patients with arterial lesions were more frequently male (91.2% vs. 62.4%, respectively; p = 0.017) and had higher rates of venous involvement (80.4% vs. 29.8%, respectively; p < 0.001) compared to patients without arterial manifestations. Thirty-nine (38.6%) patients achieved complete remission. In multivariate analysis, the presence of venous involvement (odds ratio [OR], 0.29; 95% confidence interval [CI], 0.08-1.11) and arterial occlusive lesions (OR, 0.13; 95% CI, 0.01-1.25) were negatively associated with complete remission. The use of immunosuppressants (OR, 3.38; 95% CI, 0.87-13.23) was associated with the occurrence of complete remission. The 20-year survival rate was significantly lower in BD patients with arterial involvement than in those without arterial lesions (73% vs. 89%, respectively; p < 0.0001). In conclusion, the long-term outcome of arterial lesions in BD is poor, especially in the case of occlusive lesions and associated venous involvement. The use of immunosuppressants improved the prognosis.

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Section: Discussioncontrasting

confidence: 56%

Long-Term Outcome of Arterial Lesions in Behçet Disease

et al. 2012

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“…24 The Kawasaki syndrome is a disease with an incidence of 135/100 000 children and 8-17 cases in 100 000 children , 5 years. 25 Coronary aneurysm is the main sign, but also other arterial segments can be involved. In giant cell arteritis thoracic and abdominal aneurysms are feared complications.…”

Section: Aneurysm Formationmentioning

confidence: 99%

Aortic dimensions and the risk of dissection

Erbel

Eggebrecht²

2006

Heart

177

103

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T he aorta represents a complex organ system which begins in the aortic ring adjacent to the aortic root with the origin of the two major coronary arteries, and ends at the iliac bifurcation. The subdivision into seven segments seems to be clinically important-the aortic root, the ascending aorta, the ascending aortic arch, the vessel bearing arch, the aortic isthmus, and the thoracic descending and abdominal aorta. The aorta as an organ can be regarded as a biological ''windkessel'', storing kinetic energy during systole which is delivered during diastole in order to maintain a relative constant mean aortic pressure. In particular, a high diastolic blood pressure is important for the coronary perfusion.The size of the aorta decreases with distance from the aortic valve in a tapering fashion. The normal diameter of the ascending aorta has been defined as ,2.1 cm/m 2 and of the descending aorta as ,1.6 cm/m 2 .1 The normal diameter of the abdominal aorta is regarded to be less than 3.0 cm. The normal range has to be corrected for age and sex, as well as daily workload.The aortic wall consists of three layers: intima, media, and adventitia. The intima is thin, the media contains the elastic fibres and smooth muscle cells forming a spiral layer of tissue providing the strength of the aortic wall, and the adventitia provides the nutrition with the arterial and venous vasa vasorum. An inner vasa vasorum from the aortic lumen also seems to be present.2 A wall thickness of , 4 mm is regarded as normal. AGEING cDuring life the size of the aorta increases. The normal expansion rate is about 1-2 mm/year. It involves all segments which, during childhood and in young adulthood, result in an increase of the luminal diameter of the entire aorta (figs 1 and 2). 3 In adulthood the aortic size is related to exercise and workload. The ageing of the aorta is accompanied by a loss of compliance, and an increase of wall stiffness caused by structural changes including an increase in the collagen content and formation of intimal atherosclerosis with calcium deposits.1 4 5 As a result pulse pressure is enhanced and pulse wave velocity raised, decreasing the resulting organ perfusion, particularly the predominant diastolic myocardial perfusion.Aortic sclerosis is subdivided into four stages: c intimal thickening c atheroma formation c protruding plaques c intimal flap and/or thrombus formation.Aortic sclerosis risk factors are hypertension, hyperlipidaemia, diabetes mellitus, and smoking. A thickness up to 7 mm is regarded as the upper normal tolerance limit. 1The incidence of ruptured thoracic aneurysms in individuals aged 60-69 years is about 100 cases per 10 000, in those aged 70-79 years it is about 300/10 000, and in those aged 80-89 years the incidence is 550/10 000. 6 DEFINITIONSAn enlargement of the aortic diameter exceeding at least 50% of the normal range represents an ectasia, which results in aneurysm formation when the ectasia exceeds tolerance limits. 5There are different forms of aneurysms: c true aneurysm means enlarge...

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“…Transthoracic or transesophageal echocardiography may demonstrate circumferential thickening of the aortic wall. Also, it plays a key role in the evaluation of the aortic root and valve in aortitis involving the ascending aorta associated with aortic insufficiency and aneurysm formation 7)8). In our case, echocardiography elucidated the morphological and hemodynamic changes of aortic valve, and it is clearly useful in monitoring of response of steroid therapy.…”

Section: Discussionmentioning

confidence: 64%

A Case of Idiopathic Aortitis Mimicking Severe Aortic Stenosis

Lee

Choi

2010

J Cardiovasc Ultrasound

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Aortitis is the all-encompassing pathological term ascribed to inflammation of the aorta. Regardless of the etiology, it frequently results in aortic root dilatation and aortic insufficiency rather than aortic stenosis. The rare case of aortitis such as isolated idiopathic aortitis may occur without evidence of systenic inflammatory disease or infection, and usually has subclinical nature. Even though the goals of therapy include immediate treatment of aortic inflammation or infection, the optimal management of isolated idiopathic aortitis is uncertain. We report a rare case of isolated idiopathic aortitis mimicking acute severe aortic stenosis, which was improved after steroid therapy.

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Behçet's aortitis and aortic regurgitation: a report of two cases

Cited by 26 publications

References 9 publications

Long-Term Outcome of Arterial Lesions in Behçet Disease

Long-Term Outcome of Arterial Lesions in Behçet Disease

Aortic dimensions and the risk of dissection

A Case of Idiopathic Aortitis Mimicking Severe Aortic Stenosis

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