Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

Behçet’s disease (BD) represents a multisystemic disorder that combines features of immune-mediated diseases and autoinflammatory disorders. Even though it is recognized that every type or size of vessel can be affected in this disease, there is an inability to describe a coherent model that suffici...

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Main Authors: Dimitrios Stoimenis, Nikolaos Petridis, Nikos Papaioannou
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2013/740837
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author Dimitrios Stoimenis
Nikolaos Petridis
Nikos Papaioannou
author_facet Dimitrios Stoimenis
Nikolaos Petridis
Nikos Papaioannou
author_sort Dimitrios Stoimenis
collection DOAJ
description Behçet’s disease (BD) represents a multisystemic disorder that combines features of immune-mediated diseases and autoinflammatory disorders. Even though it is recognized that every type or size of vessel can be affected in this disease, there is an inability to describe a coherent model that sufficiently explains the predilection of certain patients with BD for manifesting severe large vessel thrombosis. The inconsistent epidemiologic data and the complex genetic background of BD, along with the controversy of multiple international studies regarding the coexistence of thrombophilia in patients with BD and large vessel thrombosis, make us think that a percentage of these patients may actually suffer from a distinct clinical entity. The stimulus for this concept arose from the clinical observation of three male patients who were admitted to our clinic due to extended vena cava thrombosis. On the occasion of those clinically and laboratory resembling cases, we performed a literature review concerning the epidemiology of BD, associated thrombosis, and coexistent thrombophilic factors, in order to present some evidence, which sustains our hypothesis that certain patients with large vessel thrombosis, who share features of BD and coexistent thrombophilia, should actually be further investigated for the possibility of suffering from a distinct nosological entity.
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spelling doaj-art-c4768f70e17c4eb2b45d06ac12a831fd2025-08-20T03:23:03ZengWileyCase Reports in Medicine1687-96271687-96352013-01-01201310.1155/2013/740837740837Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?Dimitrios Stoimenis0Nikolaos Petridis1Nikos Papaioannou21st Department of Internal Medicine, “Georgios Papanikolaou” General Hospital, Exochi, 570 10 Thessaloniki, Greece1st Department of Internal Medicine, “Georgios Papanikolaou” General Hospital, Exochi, 570 10 Thessaloniki, Greece1st Department of Internal Medicine, “Georgios Papanikolaou” General Hospital, Exochi, 570 10 Thessaloniki, GreeceBehçet’s disease (BD) represents a multisystemic disorder that combines features of immune-mediated diseases and autoinflammatory disorders. Even though it is recognized that every type or size of vessel can be affected in this disease, there is an inability to describe a coherent model that sufficiently explains the predilection of certain patients with BD for manifesting severe large vessel thrombosis. The inconsistent epidemiologic data and the complex genetic background of BD, along with the controversy of multiple international studies regarding the coexistence of thrombophilia in patients with BD and large vessel thrombosis, make us think that a percentage of these patients may actually suffer from a distinct clinical entity. The stimulus for this concept arose from the clinical observation of three male patients who were admitted to our clinic due to extended vena cava thrombosis. On the occasion of those clinically and laboratory resembling cases, we performed a literature review concerning the epidemiology of BD, associated thrombosis, and coexistent thrombophilic factors, in order to present some evidence, which sustains our hypothesis that certain patients with large vessel thrombosis, who share features of BD and coexistent thrombophilia, should actually be further investigated for the possibility of suffering from a distinct nosological entity.http://dx.doi.org/10.1155/2013/740837
spellingShingle Dimitrios Stoimenis
Nikolaos Petridis
Nikos Papaioannou
Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
Case Reports in Medicine
title Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
title_full Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
title_fullStr Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
title_full_unstemmed Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
title_short Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?
title_sort behcet s disease associated large vessel thrombosis and coexistent thrombophilia a distinct nosological entity
url http://dx.doi.org/10.1155/2013/740837
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AT nikospapaioannou behcetsdiseaseassociatedlargevesselthrombosisandcoexistentthrombophiliaadistinctnosologicalentity