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Vascular Behçet's syndrome: an update
Giacomo Emmi1, Alessandra Bettiol2,3, Elena Silvestri2
1Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy. giacomaci@yahoo.it.
Internal and Emergency Medicine
|December 1, 2018
Summary
Behçet's syndrome (BS) involves vascular issues like venous and arterial thrombosis. Treatment focuses on immunosuppressants, not anticoagulants, to manage these inflammatory conditions effectively.
Area of Science:
- Vascular Medicine
- Rheumatology
- Immunology
Background:
- Behçet's syndrome (BS) is a complex vasculitis with diverse clinical manifestations.
- Vascular involvement, including venous and arterial events, is a frequent and serious complication in BS patients.
- Inflammation and coagulation pathways interplay in the pathogenesis of BS vascular complications.
Purpose of the Study:
- To review the main characteristics of vascular involvement in Behçet's syndrome.
- To focus on the clinical and therapeutic approaches for vascular manifestations of BS.
- To highlight the role of inflammatory and coagulation components in BS pathogenesis.
Main Methods:
- Literature review of Behçet's syndrome vascular involvement.
- Analysis of histological, pathogenetic, clinical, and therapeutic aspects.
- Synthesis of current understanding on inflammatory and coagulation contributions.
Main Results:
- Superficial and deep vein thrombosis affect 15-40% of BS patients; atypical thrombosis can involve major veins and sinuses.
- Arterial involvement (3-5% of patients) includes aneurysms of various arteries.
- Inflammation, particularly neutrophils, and coagulation factors drive vascular events in BS.
Conclusions:
- Effective management of BS vascular thrombosis relies on immunosuppressive therapies, not anticoagulants.
- Azathioprine, cyclosporine, and corticosteroids are indicated for venous events (DVT, SVT).
- Cyclophosphamide and anti-TNF-α agents are effective for arterial involvement and increasingly for venous events, with cerebral vein thrombosis as a potential exception for anticoagulation.