Cases reported "Behcet Syndrome"

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1/4. Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease.

    AIM: The aim of this study was to analyse the immunopathological mechanisms of vasculo-Behcet disease, which were also compared to cases of Takayasu's arteritis and inflammatory aneurysm to evaluate differences in inflammatory mechanisms. METHOD AND RESULTS: We reviewed six cases of vasculo-Behcet disease, four of Takayasu's arteritis and seven inflammatory aneurysms which underwent surgical repair. Immunohistochemical studies were performed on paraffin-embedded tissue using a labelled streptavidin-biotin method, as was in-situ hybridization for Epstein-Barr virus. Microscopically, neutrophils and lymphocytes accumulated around the vasa vasorum. neutrophils were prominent as compared to Takayasu's arteritis and inflammatory aneurysm. Elastic fibres were not severely destroyed. endothelial cells (ECs) of most vasa vasorum expressed HLA-DR. The number of vasa vasorum around which inflammatory infiltrating cells were observed in vasculo-Behcet disease was significantly greater than in inflammatory aneurysms and Takayasu's arteritis (P < 0.001). The cytokines IL-1alpha, TNF-beta and IFN-gamma were expressed in neutrophils and lymphocytes which were distributed around vasa vasorum, as well as neutrophils adherent to HLA-DR positive ECs. CONCLUSION: Our results suggest that vasculo-Behcet disease should be classified as a neutrophilic vasculitis targeting the vasa vasorum. Aneurysm formation may be related to degeneration of arterial wall caused by inflammation of the vasa vasorum.
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2/4. Thoracic aortic aneurysm associated with aortitis--case reports and histological review.

    Ten cases of post-inflammatory aneurysm of the thoracic aorta were studied morphologically using aortic wall and aortic valve tissue resected during the surgical repair of the lesions. Four cases of aortic aneurysm in Behcet's disease showed massive perivascular infiltration of the lymphocytes and plasma cells in adventitia and vasa vasorum. Fibrous obliteration of the vasa vasorum was seen, and neutrophilic infiltration was also associated in two cases with recurrent prosthetic valve failure. Three cases of aortic aneurysm associated with Takayasu's arteritis showed similar histologic features, but giant cells and histiocytic reactions were additional findings. Plasma cell infiltration was more pronounced compared to the Behcet's aortitis. One case of luetic aortitis showed a non-specific chronic inflammatory reaction with florid endothelial cell proliferation. One case of ankylosing spondylitis showed fibrous thickening at the periannular portion of the aortic valve cusps, but this finding was also seen in an aortic valve of a patient with Behcet's disease. One case without any disease association showed similar features of chronic inflammation with granuloma formation. The findings suggest that they have similar histologic features indistinguishable in terms of the clinical disease association, but a granulomatous reaction could be expected in Takayasu's arteritis.
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keywords = vasorum
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3/4. Abdominal aortic aneurysm and Behcet's disease.

    A case of abdominal aortic aneurysm with Bechcet's disease and a review of two similar cases previously reported are presented. This case and additional review of other reported major vascular complications of Behcet's disease lead us to conclude that the pathogenesis of the abdominal aneurysm is related to involvement of the aortic wall or vasa vasorum by the Behcet's disease process. Surgical treatment of this rare complication by Behcet's disease should include extra-anatomic bypass if the possibility of infection cannot be definitely excluded.
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keywords = vasorum
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4/4. Internal carotid artery dissection in a patient with Behcet's syndrome.

    The authors report a case of internal carotid artery dissection in a young woman with Behcet's syndrome. The authors postulate that a vasculitis of the vasa vasorum already suspected as the basis of aneurysm formation in course of Behcet's syndrome can account for occurrence of arterial dissection in this inflammatory condition.
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keywords = vasorum
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