Vascular Behçet’s syndrome: an update

Emmi, Giacomo; Bettiol, Alessandra; Silvestri, Elena; Scala, Gerardo Di; Becatti, Matteo; Fiorillo, Claudia; Prisco, Domenico

doi:10.1007/s11739-018-1991-y

Cited by 132 publications

(112 citation statements)

References 58 publications

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“…LEVT is the most common type of vascular involvement. On the other hand, some rare presentations of vascular affection as pulmonary artery aneurysms, cardiac involvement and BCS are associated with a significant morbidity and mortality (4,5,10).…”

Section: Discussionmentioning

confidence: 99%

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Budd-Chiari Syndrome in Behçet's Disease successfully managed with immunosuppressive and anticoagulant therapy: A case report and literature review

Oblitas

Galeano‐Valle

Toledo-Samaniego

et al. 2019

IRDR

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Behçet's Disease (BD) is a rare, chronic and recurrent inflammatory multisystemic condition of unknown origin that can affect any tissue. The vascular system is involved in 5-40% of cases of BD, including venous and arterial beds and it has a relapsing course. Budd-Chiari syndrome (BCS) is a rare complication of BD with a frequency of < 5% among patients with vascular involvement and is more frequent in men (89.5%). Two clinical presentation groups of BCS related to BD have been described: the "symptomatic" form and the "silent" form. We present a case of BD in a young woman presented as symptomatic severe BCS with rapid progression of coagulopathy reaching a spontaneous INR of 1.74 and increased ascites by ultrasound control. BD was confirmed through clinical history. The patient was treated with a high-dose pulse of corticosteroids and cyclophosphamide with a strikingly favorable response in the first forty-eight hours. Although several studies have demonstrated a survival improvement with the use of transjugular intrahepatic portosystemic shunt in patients with severe BCS, it was discarded due to the lack of evidence of this procedure in patients with BD and the fact that it could trigger a vascular pathergy phenomenon. Vascular BD should be suspected in recurrent venous and/or arterial thrombosis since it is associated with high morbidity and mortality. Immunosuppressive treatment is critical for the management of vascular involvement in BD. However, the role of anticoagulation is debatable. We suggest an algorithm for the management of BCS associated with BD.

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Section: Discussionmentioning

confidence: 99%

“…In fact, lower extremity vein thrombosis (LEVT) can be considered as the hallmark of the vascular involvement. Vascular involvement is an early manifestation of BD that affects predominantly men, and its prevalence shows a wide range (5-40%) in the published literature (1,4).…”

Section: Introductionmentioning

confidence: 99%

Budd-Chiari Syndrome in Behçet's Disease successfully managed with immunosuppressive and anticoagulant therapy: A case report and literature review

Oblitas

Galeano‐Valle

Toledo-Samaniego

et al. 2019

IRDR

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“…The vascular involvement is one of the most important in BS patients in terms of morbidity and mortality, especially for the arterial manifestations [38,39]. However, the venous involvement is certainly the most common, being the deep venous thrombosis (DVT) of the lower limbs the more typical vascular manifestation [40]. In the study by Seyahi, the diagnostic value of Doppler ultrasound (DUS) and the True Fast Imaging with Steady-state Precession (FISP) Magnetic Resonance (MRI) in the assessment of chronic DVT among male BS patients was compared [41].…”

Section: Clinical Manifestations and Phenotypesmentioning

confidence: 99%

“…The vascular involvement has been separately discussed in the review article by Emmi and colleagues [40], due to its peculiar pathogenesis and therapeutic management. Indeed, BS can be considered as the pathogenetic model of inflammation-induced thrombosis, mainly mediated by neutrophils [66][67][68].…”

Section: Treatmentsmentioning

confidence: 99%

“…In patients with vascular manifestations, the use of immunosuppressants (mainly AZA) and additional anticoagulants should be recommended at least in selected patients. Cyclophosphamide should be used for severe refractory arterial involvement, while anti-TNF-α agents represent a valid second-line treatment for both venous and arterial manifestations [40,69] .…”

Section: Treatmentsmentioning

confidence: 99%

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Behçet’s syndrome: focus on pathogenetic background, clinical phenotypes and specific treatments

Emmi

Prisco

2019

Intern Emerg Med

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Clinical characteristics and anticoagulation patterns of patients with acute pulmonary thromboembolism and hemoptysis

Li,

Xue,

Zhang

et al. 2024

Pulm. circ.

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Hemoptysis is a frequently encountered manifestation in cases of acute pulmonary thromboembolism (PTE), significantly impacting clinical decision‐making. Despite its clinical relevance, studies focusing on patients with acute PTE and hemoptysis are notably scarce. In this retrospective study, we examined data from hospitalized patients with acute PTE at Peking Union Medical College Hospital (PUMCH) between January 2012 and October 2020. Among the 896 patients analyzed, 105 (11.7%) presented with hemoptysis. Patients with hemoptysis were younger, had higher RRs, and frequently reported chest pain, predominantly showing a negative sPESI score. A significant association with autoimmune diseases was observed (39.0% vs. 16.1%; p < 0.001), along with higher occurrences of pulmonary infections (29.5%), lung cancer (21.0%), and chronic heart failure (16.2%). Hemoptysis in PTE is multifactorial; 51.4% of cases were PTE‐related, with 85.2% experiencing mild hemoptysis. Among patients with disease‐related hemoptysis (13.3%), 90.9% with massive hemoptysis had underlying diseases, predominantly lung cancer. In 35.2% of cases, the cause of hemoptysis remained undetermined, with vasculitis accounting for 29.7%. Anticoagulation strategies varied with the severity of hemoptysis; 82.9% with mild and only 27.3% with massive hemoptysis received therapeutic‐dose anticoagulation. Multivariate analysis identified massive hemoptysis as the most significant determinant of anticoagulation decisions. Patients with massive hemoptysis had the poorest outcomes, with an in‐hospital mortality rate of 36.4% and 72.7% receiving reduced or no anticoagulation.

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Vascular Behçet’s syndrome: an update

Cited by 132 publications

References 58 publications

Budd-Chiari Syndrome in Behçet's Disease successfully managed with immunosuppressive and anticoagulant therapy: A case report and literature review

Budd-Chiari Syndrome in Behçet's Disease successfully managed with immunosuppressive and anticoagulant therapy: A case report and literature review

Behçet’s syndrome: focus on pathogenetic background, clinical phenotypes and specific treatments

Clinical characteristics and anticoagulation patterns of patients with acute pulmonary thromboembolism and hemoptysis

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