Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course

Tascilar, Koray; Melikoğlu, Melike; Uğurlu, Serdal; Süt, Necdet; Çağlar, Erkan; Yazıcı, Hasan

doi:10.1093/rheumatology/keu233

Cited by 194 publications

(160 citation statements)

References 17 publications

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“…However, involvement of pulmonary arteries is two times more frequent in patients with Behçet disease who have other vascular involvement, especially deep venous thrombosis, an association which may be due to the histological properties of pulmonary arteries and their resemblance to the venous system and especially the vena cava. Therefore, pulmonary arteries are considered as a continuum of the vena cava [128]. On the other hand, lower extremity deep vein thrombosis and right-sided intracardiac thrombosis are present in 80 and 30% of patients with involvement of pulmonary arteries, respectively [129].…”

Section: Presentation and Diagnosismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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The respiratory system may be involved in all types of systemic vasculitis with varying significance and frequency. ANCA-associated vasculitis, including granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis, affects the respiratory tract more commonly than other vasculitis types. Granulomatosis with polyangiitis is always associated with upper or lower respiratory tract involvement. Pulmonary and ENT involvements are the hallmark feature of the disease and are present in 90 and 80% of cases, respectively, with frequent skin or gastrointestinal involvement. In about 10% of cases, the lung is the only organ affected. Eosinophilic granulomatosis with polyangiitis is always associated with hypereosinophilia and asthma which usually precedes the systemic manifestations by several years; however, onset of asthma and of the vasculitis may be concomitant. Parenchymal infiltrates may be migratory and rapidly resolve upon corticosteroid treatment. Diffuse alveolar hemorrhage and renal failure are typical features of microscopic polyangiitis. The former is the leading manifestation of anti-glomerular basement membrane disease and is usually part of a pulmonary-renal syndrome. Takayasu arteritis has a distinct clinical presentation due to pulmonary arteritis and may present with massive hemoptysis, chest pain, and rarely symptoms of pulmonary hypertension. Behçet disease is the most common cause of pulmonary artery aneurysm and can also cause in situ thrombosis of the pulmonary arteries. Corticosteroids and immunosuppressive agents are the mainstay of treatment. In conclusion, systemic vasculitis is a frequent cause of respiratory system involvement with diverse manifestations of distinct severity and outcome.

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Section: Presentation and Diagnosismentioning

confidence: 99%

The Respiratory System in Autoimmune Vascular Diseases

Nasser¹,

Cottin²

2018

Respiration

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“…[18][19][20][21] Cerebral venous sinus thrombosis frequently co-occurs with peripheral venous vascular events such as deep venous thrombosis and pulmonary vascular disease; therefore, serum Hcy levels are expected to be high in vascular NBS. 22 Homocysteine is known to be toxic for endothelial cells and has also been found to be associated with lower cognitive scores and neurological disorders. 23,24 There are some potential weaknesses of this study.…”

Section: Discussionmentioning

confidence: 99%

Serum Vitamin B12, Homocysteine and Methylmalonic Acid Levels in Patients With Parenchymal Neuro-Behçet’s Syndrome

et al. 2017

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Objectives: This study aims to investigate the role of serum levels of vitamin B12 (VitB12), homocysteine (Hcy), and methylmalonic acid (MMA) in the development of parenchymal neuro-Behçet's syndrome (NBS) and to compare them with healthy controls and Behçet's syndrome (BS) patients without NBS. Patients and methods: Serum VitB12, Hcy, and MMA were measured using enzyme-linked immunosorbent assay in 64 patients (36 males, 28 females; mean age 36±8.3 years; range 20 to 56 years) who met the criteria of the International Study Group for Behçet's Disease and 30 matched healthy controls (17 males, 13 females; mean age 35.76±9.6 years; range 20 to 50 years). The study participants were grouped as BS patients with NBS, BS patients without NBS, and healthy controls. Results: There was no significant difference between the groups regarding serum VitB12, Hcy, and MMA levels (p>0.05). Serum VitB12 levels were below the lower limit and serum Hcy levels were above the upper limit in all participants. MMA levels were normal in all participants. There was no correlation between the serum levels of VitB12, Hcy, and MMA and disease activity (p>0.05). Conclusion:The results of this study suggest that there is no significant association between VitB12 deficiency and parenchymal NBS. Further studies are needed to investigate the underlying mechanisms of the variable effects of VitB12 deficiency on different clinical manifestations of BS.

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“…Different types of vascular involvement tend to cluster, such as dural sinus thrombosis and pulmonary artery thrombosis. 63 Anticoagulant therapy is the primary initial intervention in these patients. Homozygous deficiency of protein C or protein S is associated with neonatal purpura fulminans, which, in some cases, includes large vessel occlusions.…”

Section: Other Disordersmentioning

confidence: 99%

How I treat catastrophic thrombotic syndromes

Ortel

Erkan

Kitchens

2015

Blood

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Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thrombocytopenic purpura (TTP) or heparin-induced thrombocytopenia (HIT), and Trousseau syndrome, but some patients present with multiple thrombotic events in the absence of associated prothrombotic disorders. Diagnostic workup must rapidly determine which, if any, of these syndromes are present because therapeutic management is driven by the underlying disorder. With the exception of atypical presentations of TTP, which are treated with plasma exchange, anticoagulation is the most important therapeutic intervention in these patients. Effective anticoagulation may require laboratory confirmation with anti–factor Xa levels in patients treated with heparin, especially if the baseline (pretreatment) activated partial thromboplastin time is prolonged. Patients with catastrophic APS also benefit from immunosuppressive therapy and/or plasma exchange, whereas patients with HIT need an alternative anticoagulant to replace heparin. Progressive thrombotic events despite therapeutic anticoagulation may necessitate an alternative therapeutic strategy. If the thrombotic process can be controlled, these patients can recover, but indefinite anticoagulant therapy may be appropriate to prevent recurrent events.

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Vascular involvement in Behçet’s syndrome: a retrospective analysis of associations and the time course

Abstract: The most common type of vascular involvement in BS is solo DVT, almost always occurring in the legs. Various forms of venous disease in BS segregate together and PAI is included in this group. EPAI segregates separately.

Cited by 194 publications

References 17 publications

The Respiratory System in Autoimmune Vascular Diseases

The Respiratory System in Autoimmune Vascular Diseases

Serum Vitamin B12, Homocysteine and Methylmalonic Acid Levels in Patients With Parenchymal Neuro-Behçet’s Syndrome

How I treat catastrophic thrombotic syndromes

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