[Spontaneous aortic isthmus aneurysm: Exceptional revelation of Behçet's disease]. / Anévrisme spontané de l'isthme aortique révélant une maladie de Behçet.

Arterial involvement in Behçet's disease is rare. The exceptional aortic isthmus location is difficult to treat. We report the case of a young man in whom an aortic isthmus aneurysm revealed Behçet's disease. In this exceptional situation, we opted for classical open surgical repair despite current preference for endovascular treatment.

[A rare localization of angio-Behçet revealed by a false aneurism of a gluteal artery treated surgically]. / Une localisation rare de l'angio-Behçet révélée par un faux anévrisme d'une artère glutéale traité chirurgicalement.

Behçet's disease is a rare chronic systemic inflammatory disease. It is more common in Turkey, North Africa and Japan. The incidence of vascular involvement (angio-Behçet) is 7 to 38%. The gluteal localization of angio-Behçet is rarely reported and could be revealed late. Aside from aneurysmal rupture, nerve compression with pain and lower limb impotence could be the main clinical signs. Because of the deep location of the lesion, management of this disease can be difficult. The classic surgical treatment remains the gold-standard especially in case of extreme urgency, non-feasibility of endovascular treatment and especially in case of associated nerve compression. We report the case of a 45-year-old man with a history of pulmonary angio-Behcet, who underwent emergency surgery for a huge false aneurism of a collateral of the hypogastric artery treated via a double anterior and posterior approach with good outcome.

Vascular Behçet's syndrome: an update.

Behçet's syndrome (BS) is a complex vasculitis, characterised by peculiar histological, pathogenetic and clinical features. Superficial venous thrombosis (SVT) and deep vein thrombosis (DVT) are the most frequent vascular involvements, affecting altogether 15-40% of BS patients. Atypical thrombosis is also an important clinical feature of BS, involving the vascular districts of the inferior and superior vena cava, suprahepatic veins with Budd-Chiari syndrome, portal vein, cerebral sinuses and right ventricle. On the other hand, arterial involvement, although affecting only 3-5% of patients, represents a unique feature of BS, with aneurysms potentially affecting peripheral, visceral and pulmonary arteries. Vascular events in BS are promoted by inflammation, with neutrophils playing a key role in the pathogenesis of thrombotic events; in turn, coagulative components such as fibrinogen, thrombin, factor Xa and factor VIIa amplify the inflammatory cascade. Understanding the contribution of inflammatory and coagulation components in the pathogenesis of BS vascular events is crucial to define the most effective therapeutic strategy. Control of vascular thrombosis is achieved with immunosuppressants drugs rather than anticoagulants. In particular, use of azathioprine and cyclosporine in association with low-dose corticosteroids should be considered in DVT and SVT cases, while treatment with cyclophosphamide together with anti-TNF-α agents can be effectively used in arterial involvement. More recently, the anti-TNF-α drugs have also been reported as a valid alternative for the treatment also of venous events, especially DVT. An exception to the use of anticoagulant in BS could be represented by cerebral veins thrombosis. In this review, we will depict the main characteristics of the vascular involvement in BS, briefly describing histological and pathogenetic features, while focusing on the clinical and therapeutical approaches of the vascular manifestations of BS.

Efficacy of anti-TNF alpha in severe and refractory major vessel involvement of Behcet's disease: A multicenter observational study of 18 patients.

OBJECTIVE: To describe the outcome and tolerance in patients treated with anti-TNFα in severe and refractory major vessel disease in Behçet's disease (BD). METHODS: A multicenter study evaluating 18 refractory BD patients with major vessel involvement [pulmonary artery (n = 4), aorta (n = 4) or peripheral artery aneurysm (n = 1) and/or pulmonary artery (n = 7), inferior vena cava (n = 5), or intra-cardiac (n = 3) thrombosis or Budd Chiari Syndrome (n = 2)] treated with anti-TNFα agents. RESULTS: Vascular remission was achieved in 16 (89%) patients. The 9 months risk of relapse was significantly higher with conventional immunosuppressants used prior anti-TNFα agents as compared to anti-TNFα therapy [OR = 8.7 (1.42-62.6), p = 0.03]. The median daily dose of corticosteroids significantly decreased at 12 months. Side effects included infection (n = 4) and pulmonary edema (n = 1). CONCLUSION: TNFα-antagonists are safe and might be associated with a decreased risk of relapse at 9 months compared to conventional immunosuppressants in BD patients with major vessels disease.

Angio-Behçet: a propósito de un caso clínico / Angio-Behçet: a case report

Se ha denominado Angio-Behçet, al conglomerado de fenómenos tromboembólicos en contexto de una Enfermedad de Behçet, que correspondería a una presentación atípica de esta patología. A veces se presenta como accidente vascular isquémico y trombosis venosa asociada a signos clásicos de Enfermedad de Behçet: Úlceras orales y genitales y uveítis. La patogénesis de esta enfermedad ha sido ampliamente estudiada en relación a posible origen genético, autoinmune o infeccioso. El tratamiento es difícil y frecuentemente tardío debido a que el diagnóstico es muy complejo y de gran reto para el clínico.

Angio-Behçet is called to the group of thromboembolic phenomena, associated to Behçet Disease and corresponds to an atypical manifestation of this disease. Sometimes it as an ischemic stroke with venous thrombosis associated to the classical signs of Behçet Disease: oral and genital ulcers and uveitis. The pathogenesis of this disease has been widely studied, with reference to a possible genetic, autoimmune or infectious origin. The treatment is difficult and is frequently late, because the diagnosis is very complex, being a great challenge for any clinician.