Fatal Thoracic Aortic Aneurysm in a Patient with Childhood-onset Vasculo-Behcet's Disease: An Autopsy Report.

Yazawa, Shogo; Ishihara, Akira; Kawasaki, Shoichiro

doi:10.2169/internalmedicine.40.1154

Cited by 6 publications

(3 citation statements)

References 6 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…15% geschätzt. Histopathologisch liegen in den meisten Fällen eine Vaskulitis (mit Über wiegen neutrophiler Granulozyten) der Vasa vasorum und konsekutive Fibrose zugrunde [18]. Wichtige Differenzialdia gnosen sind neben Lues auch die klassi schen Großgefäßvaskulitiden (Arteriitis cranialis, TakayasuArteriitis).…”

Section: Arterielle Manifestationenunclassified

Extraokuläre Manifestationen des Morbus Behçet

et al. 2012

View full text Add to dashboard Cite

Behcet's disease is a multisystem disorder with the histopathological correlate of leukocytoclastic vasculitis. The classification criteria for the disease include the presence of recurrent oral aphthous ulcers combined with at least two other manifestations, such as genital aphthous ulcers, skin manifestations (mostly erythema nodosa or pseudofolliculitis) and ocular manifestations (panuveitis or posterior uveitis with retinal vasculitis). A positive pathergy test is regarded as pathognomonic for the disease and a sterile papulopustule occurs after a sterile needle prick of the forearm. However, this test is positive in only 15% of the patients. The prognosis of Behcet's disease becomes unfavorable when vital organs are involved. This is the case for involvement of the central nervous system which occurs in 10% of patients, arterial and pulmonary arterial aneurysms and gastrointestinal involvement, which clinically and histopathologically is difficult to differentiate from inflammatory bowel disease but tends to perforate. Oligoarthritis, which occurs in approximately 50% of the patients, causes problems concerning the differential diagnosis from classical forms of spondyloarthritis. Behcet's disease is associated with HLA-B51 in 50-80% of the cases depending on the country of origin of the patient. The prognosis becomes unfavorable if the disease manifests in young male patients. The treatment of extraocular manifestations depends on the aggressiveness. Milder manifestations are treated with low dose prednisolone and steroid sparing immunosuppressants, such as azathioprine or cyclosporine A. In cases with more severe manifestations, such as central nervous system (CNS) involvement cyclophosphamide or TNF antagonists and in selected cases also interferon alpha can be considered.

show abstract

Section: Arterielle Manifestationenunclassified

Extraokuläre Manifestationen des Morbus Behçet

et al. 2012

View full text Add to dashboard Cite

show abstract

“…The most frequent location of traumatic aortic dilatation is just distal to the left subclavian artery. However, traumatic aortic aneurysm can occur in the Wiskott-Aldrich syndrome [94] Osteogenesis imperfecta [95] Behcet's syndrome [96] Homocystinuria [97,98] Relapsing polychondritis [99] Noonan syndrome [12] Idiopathic aortitis [100] Sarcoidosis [101] …”

Section: Etiologymentioning

confidence: 99%

Approach to the dilated aortic root

Boyer

Gutiérrez

Braverman

2004

Current Opinion in Cardiology

View full text Add to dashboard Cite

show abstract

“…Vasculo-BD in particular has been reported to cause arterial occlusion/stenosis and pseudoaneurysms in the aorta and its main branches. [4][5][6][7][8][9] Arterial occlusion/stenosis and pseudoaneurysms often cause ischemic organ diseases such as brain infarction 10 and life-threatening rupture, 11,12 respectively. Therefore, these arterial lesions should be detected and evaluated as soon as possible.…”

Section: Introductionmentioning

confidence: 99%

HLA-B52-positive vasculo-Beh�et disease: usefulness of magnetic resonance angiography, ultrasound study, and computed tomographic angiography for the early evaluation of multiarterial lesions

et al. 2005

View full text Add to dashboard Cite

We report a case of HLA-B52-positive Behçet disease accompanied by multiarterial lesions. A 24-year-old woman was suffering from sporadic high fever and recurrent oral and genital ulcers, and laboratory data revealed severe inflammation. A diagnosis of Behçet disease was made. Magnetic resonance angiography, ultrasound study, and computed tomographic angiography demonstrated multiarterial lesions that had caused no symptoms. These noninvasive examinations were extremely useful in evaluating asymptomatic early vascular lesions.

show abstract

Fatal Thoracic Aortic Aneurysm in a Patient with Childhood-onset Vasculo-Behcet's Disease: An Autopsy Report.

Cited by 6 publications

References 6 publications

Extraokuläre Manifestationen des Morbus Behçet

Extraokuläre Manifestationen des Morbus Behçet

Approach to the dilated aortic root

HLA-B52-positive vasculo-Beh�et disease: usefulness of magnetic resonance angiography, ultrasound study, and computed tomographic angiography for the early evaluation of multiarterial lesions

Contact Info

Product

Resources

About