Thrombosis in Behçet’s disease: a Behçet’s disease patient with complete thrombotic obstruction of IVC and both iliac veins and decreased protein S activity

Jeong, Harin; Yoo, In Kyung; Choi, Seung-Hwan; Lee, Young-Ho; Ji, Jongdae; Song, Gwan Gyu; Chung, Hwanhoon; Lee, Seunghwa; Jo, Won Min

doi:10.1007/s00296-011-2308-x

Cited by 5 publications

(3 citation statements)

References 10 publications

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“…It is a rare vasculitis in sub-Saharan Africa and is more common in the Mediterranean region. Vascular thrombosis, especially venous, is common in this condition and also constitutes a basic diagnostic criterion [ 1 ]. Its affection of the superior vena cava is rather rare with only a few cases described in the literature [ 2 , 3 ].…”

Section: Introductionmentioning

confidence: 99%

Superior vena cava syndrome revealing a Behçet’s disease

et al. 2015

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IntroductionBehçet’s disease (BD) is a rare vasculitis in sub-Saharan Africa. Vascular thrombosis, especially venous, is common in this condition and also constitutes a basic diagnostic criterion. Its affection of the superior vena cava is rather rare with only a few cases described in the literature.Case reportA 42-year-old male patient was seen at consultation presenting with a pulsatile, warm and slightly painful right latero-cervical swelling extending to the supraclavicular fossa with the presence of collateral venous circulation for three weeks prior to presentation associated with a mild headache. There were oral and genital ulcerations and erythematous skin lesions associated with a history of inflammatory recurrent arthralgia. Chest computed tomo-angiography showed cruoric internal jugular vein thrombosis extending to the superior vena cava with significant venous collateral circulation. The patient was treated with prednisolone (1 mg/kg/day) and colchicine (2 mg/day), as well as anticoagulation with heparin and vitamin K antagonist (Acenocoumarol) with regular INR monitoring. Clinical evolution was favorable during hospitalization, with residual discrete right supraclavicular swelling. There was no bleeding associated with anticoagulants use.ConclusionThe case stresses the importance of maintaining a high degree of suspicion for Behçet’s disease in all cases of venous thrombosis.

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Section: Introductionmentioning

confidence: 99%

Superior vena cava syndrome revealing a Behçet’s disease

et al. 2015

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“…Research has shown that insufficiency or a lack of natural anticoagulant proteins, including PC, PS, and antithrombin, play a role in thrombosis among BD patients (27,28). In the present case, the prolonged PT may be related to the warfarin treatment, the elevated D-dimer may be linked to using uro-kinase, and the low activity of PC and PS may have been the cause of the thrombosis event.…”

Section: Discussionmentioning

confidence: 60%

“…Similar to SLE, these results suggest that APS may be involved in BD. In relation to thrombosis, this mechanism may also have affected the present case and other cases of patients with BD, such that an occlusion or thrombosis are accompanied by elevated aCL, LA, and anti-β2-GP1 (28,36). There is no direct evidence that steroid therapy can prevent the recurrence of thrombosis in APS patients; however, it may suppress the aPL titers.…”

Section: Discussionmentioning

confidence: 76%

Anti-beta 2 glycoprotein 1 domain IgA positive antiphospholipid syndrome secondary to incomplete Behcet’s disease: a case report

Dai

Chen

et al. 2021

Ann Palliat Med

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Antiphospholipid syndrome (APS) is an autoimmune systemic disorder characterized by arterial, venous, or small vessel thrombosis, and/or recurrent early pregnancy loss, fetal loss, or pregnancy morbidity.APS is induced by persistent positive antiphospholipid antibodies (aPL), the main being lupus anticoagulant (LA), and/or anticardiolipin (aCL) antibodies, and/or anti-beta 2 glycoprotein 1 (β2-GP1) antibodies. Some studies have shown that the incidence of APS is about 5 new cases per 100,000 persons per year, and the prevalence is around 40-50 cases per 100,000 persons. APS can be primary or secondary. Secondary APS often coexists with another autoimmune disorder, most commonly systemic lupus erythematosus (SLE).Behcet's disease (BD) is usually characterized by recurrent oral and genital aphthous ulcers and ocular involvement. It can occasionally affect the venous system. BD usually affects small vessels, but can sometimes affect large veins or even a variety of veins. Because most of APS is secondary to SLE, APS secondary to incomplete BD is quite rare. This report describes a case in which a 15-year-old male experienced bilateral leg swelling and pain. The patient had a long history of self-healing recurrent mouth ulcers. Laboratory tests revealed positive β2-GP1 immunoglobulin A (IgA). His symptoms improved by using steroids, prednisolone, uro-kinase, and hirudin. In this rare case of secondary APS, the patient was diagnosed with anti-β2-GP-1 IgA positive to incomplete BD. It is a rare case of secondary APS with positive anti-β2-GP1 IgA to incomplete BD. It is suggested that patients with recurrent mouth ulcers should be closely examined to prevent thrombosis, and more laboratory markers should be used to avoid a risk of misdiagnosing patients with APS.

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