Vascular Behçet syndrome: from pathogenesis to treatment

Bettiol, Alessandra; Alıbaz-Öner, Fatma; Direskeneli, Haner; Hatemi, Gülen; Saadoun, David; Seyahi, Emire; Prisco, Domenico; Emmi, Giacomo

doi:10.1038/s41584-022-00880-7

Cited by 60 publications

(61 citation statements)

References 196 publications

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“…The BD is a rare systemic vasculitis of unknown cause which is characterized by recurrent inflammatory attacks affecting many organs [1]. Pathophysiology hallmark is an occlusive vasculitis local and systemic.…”

Section: Introductionmentioning

confidence: 99%

“…BD is observed worldwide but more frequent in countries located on the old silk road especially in the middle East, Japan, Turkey. In Africa the cases are rare essentially found in the Maghreb [1][2][3]. BD often touch young patients aged between 30 and 40 years, it is exceptional before puberty or after age of 50 year.…”

Section: Introductionmentioning

confidence: 99%

“…BD often touch young patients aged between 30 and 40 years, it is exceptional before puberty or after age of 50 year. It usually affects young subjects between 30 and 40 years old, is rare before puberty and after 50 years [1][2][3][4]. It is associated with significant morbidity and mortality particularly in men when it occurs at a very young age [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

“…It usually affects young subjects between 30 and 40 years old, is rare before puberty and after 50 years [1][2][3][4]. It is associated with significant morbidity and mortality particularly in men when it occurs at a very young age [1][2][3][4]. The main manifestations are mucocutaneous, ocular, joint, neurological, vascular (venous and arterial) and cardiac involvement [1,3,4].…”

Section: Introductionmentioning

confidence: 99%

“…It is associated with significant morbidity and mortality particularly in men when it occurs at a very young age [1][2][3][4]. The main manifestations are mucocutaneous, ocular, joint, neurological, vascular (venous and arterial) and cardiac involvement [1,3,4]. Cardiac involvement is dominated by intracardiac thrombosis, tunica involvement, coronary arteritis and aneurysms, and endomyocardial fibrosis [1,3,4].…”

Section: Introductionmentioning

confidence: 99%

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Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Ibara-Onguema

Othende

Camara

et al. 2023

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Behcet’s disease is a systemic vasculitis of the vessels for all calibers, touching arterial and venous territories. The causes of disease are unknow. BD reaches young age subjects from 10 to 45 years and affects both men and women. BD is ubiquitous but more frequent in patients from Mediterranean basin, the middle East and Asia. The diagnosis of BD is essentially clinical. The diagnostic criteria make it possible to carry the diagnosis with good sensitivity and specifity. BD evolves by recurrent inflammatory attack. BD can affect all of the organs; cardiacs manifestations are dominated by intracardiac thrombosis, the damage of three tunics, coronaryarteritis with or without myocardial infarction, coronaries aneurysms and endomyocardial fibrosis. The vascular manifestations are dominated by arterial or venous thrombosis. The presence of dilated cardiomyopathy with reduced left ventricular ejection fraction is rare. It can be explained by ischemic or inflammatory origin by cytokines. We report a case of young woman aged of 33 years to the history of 3 episodes of bipolar aphtae which presented dilated cardiomyopathy with reduced left ventricular function, biventricular thrombosis, bilateral distal pulmonary embolism with pulmonary infarction.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Ibara-Onguema

Othende

Camara

et al. 2023

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Amoebiasis

Silvestri,

Mushi,

Ngasala

2024

Vascular Damage in Neglected Tropical Diseases

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A possible role of lncRNA MEG3 and lncRNA MAFG-AS1 on miRNA 147-b in the pathogenesis of Behcet’s disease

Abobakr,

Shaker,

Hegazy

et al. 2024

Immunogenetics

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Behcet’s disease (BD) is a multisystem disease with altered Toll-like receptors (TLRs) on macrophages. Long noncoding RNA Maternally expressed gene 3 (lncRNA MEG3) and lncRNA Musculoaponeurotic fibrosarcoma oncogene family, protein G antisense 1 (MAFG-AS1) are regulators of microRNA (miRNA) 147-b, which is induced upon TLR stimulation. We included fifty BD patients, and fifty age and sex-matched controls. Real-time polymerase chain reaction (PCR) was used to measure the expression levels of serum lncRNA MEG3, lncRNA MAFG-AS1, and miRNA 147-b. LncRNA MEG3 and lncRNA MAFG-AS1 were significantly downregulated while miRNA 147-b was significantly upregulated in the BD patients' serum compared to the controls with p-value <0.001. Receiver operation characteristics (ROC) curve analysis revealed that the three biomarkers can discriminate between BD and control subjects with 76%, 100%, and 70% sensitivity respectively, and 100% specificity for all of them. There was a lower expression level of lnc RNA MEG3 among patients who had new eye involvement in the last month in comparison to those without new eye involvement (p-value=0.017). So, LncRNA MEG3, lncRNA MAFG-AS1, and miRNA147-b are promising diagnostic markers and therapeutic targets for BD patients. LncRNA MEG3 can be used as a predictor for new BD ocular involvement.

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Vascular Behçet syndrome: from pathogenesis to treatment

Cited by 60 publications

References 196 publications

Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Dilated Cardiomyopathy with Biventricular Thrombi: A Severe Manifestation of Behcet Disease

Amoebiasis

A possible role of lncRNA MEG3 and lncRNA MAFG-AS1 on miRNA 147-b in the pathogenesis of Behcet’s disease

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