Total Occlusion of Inferior Vena Cava in a Patient With Antiphospholipid Antibody Syndrome Associated With Behçet's Disease

Adamantiades–Behçet's disease (ABD) is a relapsing vasculitis of unknown aetiology and variable clinical manifestations. The syndrome can be presented in a myriad of ways and can involve nearly every organ. Although vascular involvement is not included among the ABD diagnostic criteria, it is a unique clinical manifestation in adults with a potentially devastating outcome. We report an ABD case, presenting with a thrombotic occlusion of the inferior vena cava. The authors review the recent literature, emphasizing the spectrum of vascular manifestations accompanying Behçet's disease.

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FRI0225 THE CLINICAL FEATURES AND OUTCOME OF VENA CAVA INVOLVEMENT IN BEHCET’S DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4507 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 695.1-696

Author(s):

J. Zhou ◽

J. Liu ◽

Y. Wang ◽

W. Zheng

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Inferior Vena ◽

Behçet's Disease ◽

Vena Cava ◽

Vascular Involvement ◽

Behcet's Disease ◽

Vein Thrombosis ◽

Chiari Syndrome ◽

The Mean

Background:Behcet’s disease (BD) is a systemic disease that can affect vessels of any size and type. However, only limited cases of BD patients with vena cava involvement have been reported.Objectives:To investigate the clinical features and outcome of vena cava involvement in BD patients.Methods:We retrospectively reviewed the clinical data of BD patients with vena cava involvement in our institute from August 2001 to October 2019. The treatment and outcome of these patients were also analyzed.Results:Fifty BD patients with vena cava involvement were included. The median interval between BD onset and diagnosis of vena cava involvement was 2.8 (range 0-19.4) years. Superior vena cava (SVC) involvement was detected in 22 (44.0%) patients, and 21 patients had typical manifestations of SVC syndrome. Inferior vena cava (IVC) was detected in 35 (70.0%) patients, including 7 patients diagnosed with Budd-Chiari syndrome. Seven patients had both superior and inferior vena cava involvement. Forty-five (90.0%) patients had venous involvement other than vena cava, including 19 patients with common iliac thrombosis, 12 patients with common femoral vein thrombosis, 11 patients with external iliac vein thrombosis, etc. For the other BD manifestations, oral ulceration was presented in all patients, followed by genital ulceration (35, 70.0%), Erythema nodosum (27, 54.0%) and pathergy reaction (25, 50.0%). Thirteen (26%) patients had eye involvement. Ten (20%) patients had pleural and/or pericardial effusions. Eleven (22.0%) patients had pulmonary thromboembolism, and 4 (8.0%) patients had arterial involvement. Inflammatory markers were significantly elevated in 41 (82.0%) patients when the vena cava involvement developed, the mean ESR was 34.0±29.2mm/hr, and the median CRP level was 19.9(0.2-177.0) mg/L. The mean BDCAF2006 score of all patients was 4.6±1.6. Glucocorticoid was used in 47 (94.0%) patients after vena cava involvement was diagnosed, and cyclophosphamide was the first-choice immunosuppressant. Forty-one (82.0%) patients received anticoagulation treatment. Five patients had received placement of IVC filter, and 3 patients had taken balloon dilation of IVC. With a mean follow up of 4.1±3.8 years, 45 patients (90.0%) achieved clinical improvements, 6 patients (12.0%) had relapse of vascular involvement, 5 patients (10.0%) died. The respective estimated cumulative 1- and 5- years relapse-free rates were 90.9% and 83.1%, and the respective estimated 1- and 5- years survival rates were 95.9% and 90.1%.Conclusion:Vena cava involvement is a rare complication in BD patients. The prognosis of these patients is relatively optimistic after proper treatment. To the best of our knowledge, our study is the largest cohort of BD patients with vena cava involvement.References:[1]Y. Fei, X. Li, S. Lin et al. Major vascular involvement in Behçet’s disease: a retrospective study of 796 patients. Clinical Rheumatology, 2013, 32 (6): 845–852.[2]Seyahi E, Caglar E, Ugurlu S et al. An outcome survey of 43 patients with Budd-Chiari syndrome due to Behçet’s syndrome followed up at a single, dedicated center. Semin Arthritis Rheum, 2015, 44(5):602-609.Acknowledgments:We gratefully thank all the patients who participated in our study.Disclosure of Interests:None declared

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THU0599 PULMONARY ARTERY ANEURYSM, BUDD CHIARI SYNDROME, INTRACARDIAC AND INFERIOR VENA CAVA THROMBOSES: AN UNUSUAL CASE OF BEHÇET’S DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6668 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 541-542

Author(s):

S. Ousalem ◽

S. Beaudoin

Keyword(s):

Pulmonary Artery ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Vena Cava ◽

Artery Aneurysm ◽

Vascular Involvement ◽

Budd Chiari Syndrome ◽

Behcet's Disease ◽

Chiari Syndrome

Background:Behçet’s disease (BD) or “Silk Road” disease is a rare multisystemic inflammatory disease of unknown etiology.Vascular involvement manifested as thrombosis, arterial aneurysm, and occlusion can carry a high mortality risk. BD can be a diagnostic conundrum with its broad array of clinical presentations.Objectives:Identifying vasculo-Behçet’s disease and its management.Methods:A 25-year-old man born in Malaysia and known for cirrhosis due to idiopathic Budd Chiari syndrome presented to the emergency room with a transient ischemic attack. An inferior vena cava (IVC) occlusive thrombus and a patent foramen ovale (PFO) were discovered. Thrombolysis, angioplasty, PFO closure, and a transjugular intrahepatic portosystemic shunt (TIPS) procedure were performed. The following year, the patient experienced numerous IVC and TIPS-associated thromboses as well as a right atrial thrombus attached to his PFO closure device, all of which were refractory to anticoagulation. A few months later, the patient suffered from an acute right anterior cerebral artery stroke, with no etiology uncovered at the time. It was later determined that the patient had experienced years of recurrent oral and genital aphthae, thereby prompting a strong clinical suspicion of BD. Six months later, after only one appointment at the rheumatology clinic during which he was prescribed colchicine, the patient presented to the hospital with hemoptysis. A computed tomography (CT) pulmonary angiogram revealed a right lower lobar pulmonary arterial aneurysm with a peripheral thrombus, a right bronchial artery dilatation, and pulmonary emboli. The patient declined anticoagulation and was sent home. Two months later, he returned to the hospital, this time with hematemesis. A repeat CT pulmonary angiogram was performed and showed an increasing pulmonary emboli burden and an enlarging aneurysm. A thrombophilia workup was negative.Results:A diagnosis of BD with pulmonary aneurysms was made and treatment was initiated with methylprednisolone pulses and monthly intravenous cyclophosphamide as recommended by the European League Against Rheumatism. A month later, there was radiological evidence of significant improvement in the burden of pulmonary emboli, an interval decrease in the aneurysm’s diameter, and resolution of the right atrial thrombus.Conclusion:BD with vascular involvement or vasculo-Behçet’s disease can affect small, medium, and large vessels of both the venous and arterial vasculatures and is thought to originate from vessel wall inflammation.Thrombi in vasculo-Behçet’s disease are typically quite adherent to the vessel walls and tend not to embolize. In this case, pulmonary arterial thrombosis burden was significantly decreased after immunosuppression alone, favoring a diagnosis of in situ thrombosis rather then thromboembolism. Moreover, pulmonary artery aneurysm, Budd-Chiari syndrome, and vena cava thrombosis, which are quite uncommon and carry the highest mortality risk in vasculo-Behçet’s, were all present in this case. Early recognition can be life-saving as immunosuppression is the first-line therapy rather than anticoagulation, which carries a significant risk of pulmonary hemorrhage in the presence of a pulmonary artery aneurysm.References:[1]Seyahi, E., Behcet’s disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol, 2016. 30(2): p. 279-295.[2]Hamuryudan, V., et al., Pulmonary artery aneurysms in Behcet syndrome. Am J Med, 2004. 117(11): p. 867-70.[3]Kobayashi, M., et al., Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease. Histopathology, 2000. 36(4): p. 362-71.[4]Seyahi, E. and S. Yurdakul, Behcet’s Syndrome and Thrombosis. Mediterr J Hematol Infect Dis, 2011. 3(1): p. e2011026.[5]Hatemi, G., et al., 2018 update of the EULAR recommendations for the management of Behcet’s syndrome. Ann Rheum Dis, 2018. 77(6): p. 808-818Disclosure of Interests:None declared

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