FRI0225 THE CLINICAL FEATURES AND OUTCOME OF VENA CAVA INVOLVEMENT IN BEHCET’S DISEASE
Authors: J. Zhou, J. Liu, Y. Wang, W. Zheng
Journal: Annals of the Rheumatic Diseases (2020)

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