BEHÇET’S SYNDROME AND THROMBOSIS

Behçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatenin...

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Main Authors: Emire Seyahi, Sebahattin Yurdakul
Format: Article
Language:English
Published: PAGEPress Publications 2011-07-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
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Online Access:http://www.mjhid.org/index.php/mjhid/article/view/282
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author Emire Seyahi
Sebahattin Yurdakul
author_facet Emire Seyahi
Sebahattin Yurdakul
author_sort Emire Seyahi
collection DOAJ
description Behçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40 % of cases.  BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitutional symptoms and increased acute phase response and is the major cause of increased mortality.  A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks.
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spelling doaj-art-e34fc6c89ec549dfa27270cf93fcf7b42025-08-20T02:50:41ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062011-07-0131e2011026e201102610.4084/mjhid.2011.026174BEHÇET’S SYNDROME AND THROMBOSISEmire SeyahiSebahattin YurdakulBehçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40 % of cases.  BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitutional symptoms and increased acute phase response and is the major cause of increased mortality.  A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks.http://www.mjhid.org/index.php/mjhid/article/view/282Behçet’s syndrome, vasculitis, venous thrombosis, lower extremity vein thrombosis, vena cava thrombosis, azathioprine
spellingShingle Emire Seyahi
Sebahattin Yurdakul
BEHÇET’S SYNDROME AND THROMBOSIS
Mediterranean Journal of Hematology and Infectious Diseases
Behçet’s syndrome, vasculitis, venous thrombosis, lower extremity vein thrombosis, vena cava thrombosis, azathioprine
title BEHÇET’S SYNDROME AND THROMBOSIS
title_full BEHÇET’S SYNDROME AND THROMBOSIS
title_fullStr BEHÇET’S SYNDROME AND THROMBOSIS
title_full_unstemmed BEHÇET’S SYNDROME AND THROMBOSIS
title_short BEHÇET’S SYNDROME AND THROMBOSIS
title_sort behcet s syndrome and thrombosis
topic Behçet’s syndrome, vasculitis, venous thrombosis, lower extremity vein thrombosis, vena cava thrombosis, azathioprine
url http://www.mjhid.org/index.php/mjhid/article/view/282
work_keys_str_mv AT emireseyahi behcetssyndromeandthrombosis
AT sebahattinyurdakul behcetssyndromeandthrombosis