Vascular Behçet syndrome: from pathogenesis to treatment.

Autor: Bettiol A; Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.; Internal Interdisciplinary Medicine Unit, Behçet Center, Careggi University Hospital, Firenze, Italy., Alibaz-Oner F; Vasculitis Clinic, Marmara University, School of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey., Direskeneli H; Vasculitis Clinic, Marmara University, School of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey., Hatemi G; Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, School of Medicine, Istanbul, Turkey.; Behçet's Disease Research Centre, Istanbul University-Cerrahpasa, Istanbul, Turkey., Saadoun D; Sorbonne Universités AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Département de Médecine Interne et Immunologie Clinique, F-75013, Paris, France.; Centre National de Références Maladies Autoimmunes Systémiques Rares, Centre National de Références Maladies Autoinflammatoires et Amylose Inflammatoire; Inflammation-Immunopathology-Biotherapy Department (DMU 3iD); INSERM 959, Groupe Hospitalier Pitie-Salpetriere, AP-HP, Paris, France., Seyahi E; Division of Rheumatology, Department of Internal Medicine, Istanbul University-Cerrahpasa, School of Medicine, Istanbul, Turkey.; Behçet's Disease Research Centre, Istanbul University-Cerrahpasa, Istanbul, Turkey., Prisco D; Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy.; Internal Interdisciplinary Medicine Unit, Behçet Center, Careggi University Hospital, Firenze, Italy., Emmi G; Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy. giacomo.emmi@unifi.it.; Internal Interdisciplinary Medicine Unit, Behçet Center, Careggi University Hospital, Firenze, Italy. giacomo.emmi@unifi.it.; Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Clayton, Victoria, Australia. giacomo.emmi@unifi.it.
Jazyk: angličtina
Zdroj: Nature reviews. Rheumatology [Nat Rev Rheumatol] 2023 Feb; Vol. 19 (2), pp. 111-126. Date of Electronic Publication: 2022 Dec 21.
DOI: 10.1038/s41584-022-00880-7
Abstrakt: Behçet syndrome is a rare, chronic inflammatory disease of unknown aetiopathogenesis, most commonly presenting with mucocutaneous and ocular manifestations. Vascular involvement, most frequently superficial vein and deep vein thrombosis, can occur in up to 50% of patients with Behçet syndrome. Venous thrombosis at atypical sites (inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right atrium and/or ventricle) and arterial involvement (mostly in situ thrombosis and aneurysms of the pulmonary arteries, as well as aneurysms of the abdominal aorta, and peripheral and visceral arteries) are also unique features of Behçet syndrome. Behçet syndrome is considered a natural model of inflammation-induced thrombosis in humans, with an impaired immune-inflammatory response rather than traditional cardiovascular risk factors contributing to thrombogenesis. Specifically, neutrophil hyperactivation and neutrophil-mediated mechanisms of damage directly promote endothelial dysfunction, platelet activation and thrombogenesis in Behçet syndrome. This unusual pathogenesis directly determines the treatment approach, which relies mostly on immunosuppressants rather than anticoagulants for treatment of thrombosis and for secondary prevention. This Review discusses the main histopathological, pathogenetic and clinical aspects of vascular Behçet syndrome, addressing their implications for therapeutic management. Future perspectives in terms of pathogenetic studies, disease monitoring and treatment strategies are also discussed.
(© 2022. Springer Nature Limited.)
Databáze: MEDLINE
Externí odkaz: