A review of current management of vasculo-Behcet's

Merashli, Mira; Eid, Rozana El; Uthman, Imad

doi:10.1097/bor.0000000000000458

Cited by 29 publications

(27 citation statements)

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“…A skin biopsy also revealed histological findings of necrotizing vasculitis. Dilatation of the SMA and its jejunal branch led to a diagnosis of vasculo-BD (10).…”

Section: Case Reportmentioning

confidence: 99%

“…The arterial complications are commonly isolated, but can be multiple (8), with the abdominal aorta as the most common site (9). BD lesions of superior mesenteric artery (SMA) are very rare, but can result in arterial rupture and death (5,10). Although the general epidemiological features of vascular manifestations in patients with BD have been reported (11), the features of SMA lesions have not fully been summarized.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Superior Mesenteric Artery Vasculitis in Behçet's Disease: A Case Report and Literature Review

Kakehi¹,

Adachi²,

Fukuyasu³

et al. 2019

Intern. Med.

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A 55-year-old male presented with abdominal pain that had begun about 5 days ago. Physical examination revealed oral aphtha, genital aphthosis, and pseudofolliculitis, and the patient was diagnosed with incomplete Behçet's disease (BD). Contrast-enhanced computed tomography (CECT) showed dilation of the superior mesenteric artery and mesenteric infiltration of inflammation, indicating vasculo-BD. The symptoms were improved by 3-day of intravenous methylprednisolone pulse therapy followed by oral prednisolone. A literature review suggested that vasculo-BD should be included as a differential diagnosis in cases with unexplained abdominal pain, arterial dilation, and mesenteric invasion, and CECT examination and steroid therapy should be considered.

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“…A skin biopsy also revealed histological findings of necrotizing vasculitis. Dilatation of the SMA and its jejunal branch led to a diagnosis of vasculo-BD (10).…”

Section: Case Reportmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Superior Mesenteric Artery Vasculitis in Behçet's Disease: A Case Report and Literature Review

Kakehi¹,

Adachi²,

Fukuyasu³

et al. 2019

Intern. Med.

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Section: Discussionmentioning

confidence: 99%

“…Conceptually, in a genetically susceptible host, Behcet’s vasculitis causes endothelial damage that induces a hypercoagulable state augmented by increased platelet aggregation and impaired fibrinolysis 8. Furthermore, T-cell overproduction of proinflammatory interleukin (IL)-21 and IL-17A intensifies the inflammatory cascade, with other ILs and TNF playing a role 8. Arterial aneurysmal formation in Behcet’s is not atherosclerotic but due to arterial wall inflammation causing damage to the media, fibrosis, obliteration of the vasa vasorum and, ultimately, wall dilation with pseudoaneurysm formation 9.…”

Section: Discussionmentioning

confidence: 99%

Coeliac artery dissection as a rare manifestation of Behcet’s disease

Ocon¹,

Mehta

Peredo-Wende

2018

BMJ Case Reports

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Behcet's disease is a multisystemic vasculitis. Arterial involvement in the form of acute dissection is rare. A 42-year-old Lebanese man with Behcet's disease presented with severe abdominal pain. On exam, blood pressure was 162/104 mm Hg, and he exhibited epigastric tenderness. CT angiogram demonstrated an acute dissection of the coeliac artery trunk, common hepatic artery and proper hepatic arteries, with asymmetric thickening of the proximal left subclavian artery and circumferential thickening of the abdominal infrarenal aorta suggestive of vasculitis. Treatment included intravenous clevidipine, nitroprusside and methylprednisolone, which transitioned to oral metoprolol, amlodipine and prednisone. He responded well. Arterial dissections have been described with Behcet's. We report a coeliac artery aneurysm in association with a flare of Behcet's disease. Arterial wall inflammation combined with the sheering forces of hypertension likely predisposes to arterial dissection.

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“…The management of BD depends on whether there are self-limiting manifestations or serious organ involvement, and should be individualised according to age, gender and type. 12,13 In systemic severe disease, intravenous pulse corticosteroids followed by daily oral doses, azathioprine, cyclophosphamide, cyclosporine-A, methotrexate, mycophenolate mofetil, tacrolimus, interferon (IFN)-α or TNF-α inhibitors (etanercept, infliximab) can be chosen. 12 A new oral inhibitor of phosphodiesterase-4, apremilast, has been studied in patients without major organ involvement and demonstrated a complete response in significantly more patients.…”

Section: Treatment Optionsmentioning

confidence: 99%

Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

Özyurt¹,

Sfikakis²,

Síva³

et al. 2018

European Neurological Review

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Background:Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used.Objectives:To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium.Data sources:Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews.Limitations:The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review.Conclusions:Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.

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A review of current management of vasculo-Behcet's

Abstract: Behcet disease patients are at risk of developing multiple vascular complications including thrombosis and aneurysms. Treatment should focus on reducing inflammation; and the role of anticoagulation is still debatable.

Cited by 29 publications

References 50 publications

Superior Mesenteric Artery Vasculitis in Behçet's Disease: A Case Report and Literature Review

Superior Mesenteric Artery Vasculitis in Behçet's Disease: A Case Report and Literature Review

Coeliac artery dissection as a rare manifestation of Behcet’s disease

Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

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