Background/Purpose: Venous thrombosis (VT) is a serious and potentially life-threatening manifestation of Behçet disease (BD). However, there is little evidence of the management of the VT in BD. To review the treatment used in venous thrombotic events in BD.

Methods: A systematic review was performed according to the PICO approach. Several synonyms for the main components (i.e. Behçet, venous thrombosis, treatment) were used. The literature search was performed in Medline and Embase from databases inception to 1st November 2017. Only articles in English and Latin languages were retained. We excluded abstracts, reviews or letters. From the selected studies, data about the VT, treatment and treatment response were retired using a predefined data collection form.

Results: The literature search resulted in 1552 articles (figure 1). The main reason for article exclusion after full-text review was the lack of description of VT and its treatment. Finally, 26 articles were included in the analysis with a total of 1899 patients. Mean age (standard deviation, SD) was 32.8 (5.6) years and most of them were men (77.7%). The mean (SD) duration time between disease onset and VT onset was evaluated in only 16 articles and was 4.67 (2.9) years.

Superficial VT was evaluated in 34.6% articles, involving a number of 29 patients, deep VT in 76.9% articles and 291 patients, cerebral VT in 40.7% articles and 140 patients, inferior or superior cava vein VT in 73.1% articles and 270 patients and Budd-Chiari syndrome in 53.8% articles and 73 patients.

Details of the reported treatments are depicted in table 1. Due to the lack of detailed treatment description, no relationship between the type of treatment and the type of VT could be established. Treatment response was evaluated in 23 (88.5%) articles. A good treatment response, i.e., the thromb resolution was reported in 11 articles, corresponding to 161 patients. Death due to VT was reported in 10 articles (38.5%) and 24 patients. Complications and lack of treatment response were also reported: recurrences, extension or progression of the thromb (11 articles, 450 patients), aneurisms (6 articles, 28 patients), new thrombs (3 articles, 20 patients), post thrombotic complications (3 articles, 38 patients). However, data regarding treatment response were inconstantly reported and the outcomes used for assessing it were very heterogeneous. Therefore, correlations and predictive factors of complications or mortality could not be identified.  

Conclusion: There is a high variability in reporting treatment of VT related to BD with respect to type, indication and evaluation of response.