Thrombosis in Behcet's disease: Report of a case followed by a systematic review using the methodology of evidence-based medicine

Background: Behcet's disease (BD) is a rare systemic inflammatory disease o f unknown aetiology with a variety of organ manifestations. Methods: A case of Behcet's disease complicated by deep vein thrombosis (DV T) and inferior vena cava thrombosis referred to our institution is reporte d. In addition, a structured literature search using the methodology of evi dence-based medicine for the localization of venous lesions, treatment moda lities, and outcome of patients with Behcet's disease and concurrent thromb osis was performed. A modified validity score was assigned by established m ethods. Results: In total, 214 citations were identified using our search strategy. Among these citations, 10 papers including a total of 32 patients (25 male , 7 female) met the inclusion criteria and were incorporated into this over view together with our patient. All studies consisted of serial case report s without control subjects. Neither localization of venous lesions, nor tre atment modalities were homogenous not allowing general recommendations. Conclusions: No data are available from controlled studies regarding treatm ent modalities of patients presenting with Behcet's disease complicated by concurrent thrombosis. This lack of evidence implicates the need for large scale and co-ordinated registries including data on the acute treatment as well as the prevention of future thrombotic events in this clinical setting . The diagnostic criteria of the "International Study Group for Behcet's Di sease" may well serve as a basis for this approach.