Arterial and venous involvement in Behçet’s syndrome: a narrative review

Toledo-Samaniego, Neera; Oblitas, Crhistian-Mario; Peñaloza-Martínez, Eduardo; Del-Toro-Cervera, Jorge; Álvarez‐Sala‐Walther, Luis Antonio; Demelo‐Rodríguez, Pablo; Galeano‐Valle, Francisco

doi:10.1007/s11239-022-02637-1

Cited by 22 publications

(19 citation statements)

References 61 publications

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“…Venous thrombosis occurs in a range of 15 to 45% of affected patients, predominantly as superficial and deep vein thrombosis. Various studies have affirmed that up to 30% of cases may occur as major venous thrombosis (superior and inferior vena cava (SVC and IVC), portal vein, hepatic vein, and dural sinuses) [ 61 , 62 ]. The pathogenesis of thrombosis in BD is due to both vasculitic inflammatory phenomena of the large veins and hypercoagulability status [ 63 ].…”

Section: Imaging Approach To Thoracic Involvement In Behcet’s Diseasementioning

confidence: 99%

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

et al. 2022

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Behcet’s disease (BD) is a chronic systemic inflammatory disorder characterized by underlying chronic vasculitis of both large- and small-caliber vessels. Thoracic involvement in BD can occur with various types of manifestations, which can be detected with contrast-enhanced MSCT scanning. In addition, MR can be useful in diagnosis. Characteristic features are aneurysms of the pulmonary arteries that can cause severe hemoptysis and SVC thrombosis that manifests as SVC syndrome. Other manifestations are aortic and bronchial artery aneurysms, alveolar hemorrhage, pulmonary infarction, and rarely pleural effusion. Achieving the right diagnosis of these manifestations is important for setting the correct therapy and improving the patient’s outcome.

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Section: Imaging Approach To Thoracic Involvement In Behcet’s Diseasementioning

confidence: 99%

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

et al. 2022

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“…Platelet activation mediated by cytokine production and neutrophil activation contributes to inflammatory thrombi formation. 13 As a result of a unique cell death mechanism known as NETosis, which occurs under inflammatory or infectious conditions, neutrophils can produce neutrophil extracellular traps (NETs). Furthermore, NETs have been linked to the maintenance of inflammation and the vascular damage seen in BD.…”

Section: Discussionmentioning

confidence: 99%

Lesion probability map in cerebral vein thrombosis due to Behçet's disease

et al. 2022

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Aim Behçet's disease (BD) is a multisystemic inflammatory disease. Cerebral venous sinus thrombosis (CVST) is the second most common form of neuro‐BD after parenchymal central nervous system involvement. The purpose of this study was to construct flow‐void probability maps of patients with CVST with and without BD to visually illustrate the impacted cerebral venous sinuses, to compare the subgroups of patients, and investigate the effect of thrombus localization on clinical findings. Methods Seventeen patients with a diagnosis of BD‐related CVST (CVST‐BD) and 23 patients with a diagnosis of CVST related to other etiologies (CVST‐O) were included. We collected data including gender, age at onset of BD and CVST, presenting symptoms, neurological findings, and the etiology. High‐resolution magnetic resonance venographies obtained during CVST were used to mark and digitalize thrombosed areas. Thrombus probability and subtraction maps were created to reveal the differences between the subgroups. Results Remarkably, all patients with CVST‐BD had thrombosis in the transverse sinus (TS). However, TS was affected in 73.9% of the CVST‐O patients (17/17 in CVST‐BD vs 17/23 in CVST‐O, P = .03). Thrombosis developed mostly in the superior sagittal sinus (SSS) and TS in the CVST‐O group (11/23, 47.8% and 17/23, 73.9%, respectively). The frequency of SSS thrombosis tended to be higher in the CVST‐O (47.8% vs 23.5%, P = .19). Conclusion Venous infarction and hemorrhage were less common in patients with CVST‐BD. The only clinical symptom in most of the CSVT patients with BD was headache due to elevated intracranial pressure. TS thrombosis was more common in patients with BD.

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“…Vascular involvement is a major contributor to disability and mortality rates of BD patients. Therefore, early diagnosis and treatment of vascular lesions are very important for prognosis of BD patients ( 40 ). Currently, diagnosis of BD depends on clinical manifestations, however there are no specific pathological or laboratory tests to help diagnose BD.…”

Section: Discussionmentioning

confidence: 99%

Case report and analysis: Behçet’s disease with lower extremity vein thrombosis and pseudoaneurysm

Wang

Zhang

et al. 2022

Front. Immunol.

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BackgroundBehçet’s disease (BD) is a unique autoimmune chronic systemic vasculitis that affects veins and arteries of all sizes. BD can lead to recurrent vascular events, especially venous thrombosis, with an incidence rate of 40%, or pseudoaneurysms formed under long-term inflammatory reaction or iatrogenic stimulation. BD-related risk factors promote endothelial dysfunction, platelet activation and overactivation of tissue factors leading to mural inflammatory thrombi. Thrombosis may be the first clinical manifestation of BD.Case presentationA 32-year-old man complaining of progressive swelling and pain in the right lower extremity for 30 days was initially diagnosed with “venous thrombosis of the right lower extremity,” using color Doppler ultrasonography. Patient underwent inferior vena cava filter placement combined with deep vein angioplasty of the right lower extremity and catheter-directed urokinase thrombolysis. Postoperative oral anticoagulant therapy was administered. However, the patient was readmitted 20 days later for pulsatile pain in the right groin. Prior medical history included 4 years of repeated oral and perineal ulcers, and 2 months of blurred vision. Abdominal computed tomography angiography (CTA) revealed rupture of the right common iliac artery (CIA) and left internal iliac artery (IIA), complicated by a pseudoaneurysm. Based on the clinical manifestations and other auxiliary examination results, the patient was re-diagnosed with “BD combined with deep venous thrombosis of the right lower extremity and an iliac artery pseudoaneurysm.” Stent implantation was performed for iliac artery pseudoaneurysm after symptoms were controlled with timely immunosuppressive therapy. After endovascular treatment, the patient underwent continued immunosuppressive therapy and dynamic reexaminations of abdominal CTA, which revealed that a small amount of contrast agent at the stent in the right CIA continued to flow into the cavity of the pseudoaneurysm; in addition, the size of the pseudoaneurysm was gradually increasing. Therefore, the patient underwent a second stent implantation for iliac artery pseudoaneurysm, and the condition improved further.ConclusionThe importance of early diagnosis of BD should be recognized, and the choice of interventional and surgical procedures should be carefully evaluated, as this may trigger further damage to vascular access in BD patients with aneurysm.

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Arterial and venous involvement in Behçet’s syndrome: a narrative review

Cited by 22 publications

References 61 publications

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

Behçet’s Disease: A Radiological Review of Vascular and Parenchymal Pulmonary Involvement

Lesion probability map in cerebral vein thrombosis due to Behçet's disease

Case report and analysis: Behçet’s disease with lower extremity vein thrombosis and pseudoaneurysm

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