Compelling nature of arterial manifestations in Behçet disease

Işcan, Zafer; Vural, Kerem M.; Bayazit, Murat

doi:10.1016/j.jvs.2004.09.018

Cited by 105 publications

(83 citation statements)

References 13 publications

(16 reference statements)

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“…Fatal vascular complications are frequent; however, debate about the optimal time point for surgery continues (1). Iscan et al reported a mortality of 2 patients of 34 operations in a total of 20 patients and a 5-year repeat operation-free survival rate of 26% (13). Our findings show that disease activity should be controlled before surgery is performed and immunosuppressive treatment should rather be increased than reduced.…”

Section: Discussionmentioning

confidence: 55%

Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis

Adler¹,

Baumgartner²,

Villiger³

2012

Arthritis Care & Research

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Objective. To evaluate the therapeutic effect of infliximab in patients with inflammatory vascular lesions due to Behçet's disease (BD). Methods. Seven patients with clinical evidence of severe vascular BD were analyzed: 3 patients with aortic involvement, 1 with recurrent venous thrombosis of the pelvic veins, 1 with recurrent venous and arterial thromboses of the thigh, and 2 with retinal vasculitis. Infliximab was initiated with 3-5 mg/kg of body weight and infusions were repeated in intervals of 4 weeks as either first-line therapy in 3 patients or add on after failure of conventional immunosuppression in the remaining 4. Ongoing immunosuppression consists of a various combination of azathioprine (n ‫؍‬ 2), methotrexate (n ‫؍‬ 3), cyclosporine (n ‫؍‬ 3), and low-dose glucocorticoids (n ‫؍‬ 3). Results. Control of inflammation was seen 1-5 days after infliximab induction in all patients. C-reactive protein level was reduced from a mean of 89 mg/liter prior to infliximab to 9 mg/liter thereafter. Vision increased rapidly in patients with retinal vasculitis. Vascular grafts remained patent. The inflamed and dissected aortic wall healed over a period of 6 months. Infliximab could be stopped in 2 patients; intervals could be extended in 4 to a maximum of 8 weeks. Infliximab and basic immunosuppression were well tolerated; no drug-induced side effects were recorded. Conclusion. Infliximab is effective in inducing and maintaining remission of vasculitic activity in patients with BD. The rapid effect together with excellent tolerability suggests that infliximab should be considered as a first-line agent in severe vascular BD.

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Section: Discussionmentioning

confidence: 55%

Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis

Adler¹,

Baumgartner²,

Villiger³

2012

Arthritis Care & Research

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“…Patients with Behçet's disease may develop pulmonary involvement in up to 7% of the cases [10]. The most common complication is a pulmonary aneurysm, which in some cases gives rise to massive hemoptysis [11]. Other complications include arterial and venous thromboses, recurrent pneumonia, and arteriobronchial fistulas [12].…”

Section: Discussionmentioning

confidence: 99%

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

Firouzi¹,

Behrooj²,

Pirborj³

et al. 2017

J Cardiovasc Dis Diagn

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Multiple Pulmonary Arteriovenous Fistulas (PAVFs) are rare vascular abnormalities in the general population, with a report on 15000 autopsies revealing only 3 cases of multiple PAVFs. However, these abnormal communications between pulmonary arteries and veins are commonly seen in patients with Hereditary Hemorrhagic Telangiectasia (HHT). Several conditions such as cirrhosis, chest trauma, metastatic carcinoma, schistosomiasis, and systemic amyloidosis are accountable for acquired form PAVF. We describe 2 patients (mother and son) with multiple PAVFs and no apparent signs or symptoms suggestive of HHT. A pulmonary artery injection wes performed for Case 1 (mother) and showed bilateral PAVFs with aneurysmal dilation. Given the diagnosis of PAVFs in the mother, we considered the same pathology for the child's symptoms. Therefore, the boy also underwent pulmonary angiography, which revealed multiple bilateral PAVFs. Both patients underwent interventional angiography and the PAVFs were occulted with stainless coil. In conclusion, the presence of multiple PAVFs should be considered in the differential diagnosis of patients with unexplained exertional dyspnea and cyanosis, even in the absence of diagnostic features suggestive of HHT.

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“…Thrombi may develop within the aneurysmal sac, and very rarely distal embolization may occur leading to threatening limb ischemia [74]. The femoral artery is frequently affected.…”

Section: Occlusive Lesionsmentioning

confidence: 99%

Vascular Manifestations of Behçet’s Disease

Demïrtürk¹,

Tünel²,

Alemdaroğlu³

2017

Behcet's Disease

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Behçet's disease (BD), a very morphologically diverse systemic disease, may involve the vascular system. The venous system is the most frequently attacked vessel system.The arterial system, when involved, increases the severity and morbidity of Behçet's disease. Cardiac involvement, although rare, can be very subtle and in itself increases the mortality. Vasculitis is the hallmark pathology resulting in occlusion, aneurysms, or both.Vascular involvement may be very challenging in all phases of treatment beginning from diagnosis till recovery and remission.

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Compelling nature of arterial manifestations in Behçet disease

Cited by 105 publications

References 13 publications

Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis

Behçet's disease: Successful treatment with infliximab in 7 patients with severe vascular manifestations. A retrospective analysis

Multiple Pulmonary Arteriovenous Fistula in a Mother and His Son: A Case Report

Vascular Manifestations of Behçet’s Disease

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