Intracardiac thrombosis in Behçet's Disease successfully treated with immunosuppressive agents: A case of vascular pathergy phenomenon

Abstract

Behçet's Disease (BD) is a rare multi-systemic inflammatory disorder classified as a systemic vasculitis of unknown aetiology. Vascular involvement occurs in approximately 5-51.6% cases, affecting venous and arterial vessels. Cardiac involvement is rare in BD (6%). There have been published approximately 93 cases of BD associated with intracardiac thrombosis, with different treatments and courses. We present a case of a 35-year-old spanish male that, after a percutaneous pharmacomechanical thrombectomy with venous stent placement, developed high fever and extensive venous thrombosis despite anticoagulation including intracardiac thrombosis (ICT) in the right ventricle and pulmonary embolism that leaded to the diagnosis of BD. The patient was successfully treated with immunosuppressants, achieving the complete resolution of ICT. We hypotesize that the endovenous procedure could have acted as a trigger for the posterior acute attack of the disease, representing a 'vascular pathergy phenomenon'. Vascular BD has to be suspected in cases of thrombosis recurrence despite correct anticoagulation, and intense immunosuppressive treatment should be considered.

Keywords: Behçet disease; immunosuppressive agents; intracardiac thrombus; thrombectomy; venous thromboembolism.

Figure 1.

(A), Positron Emission Tomography - Computed Tomography (PET-CT) shows thrombosis of the stent in the left common iliac vein with two areas of hyper-metabolic activity (white arrows); (B), PET-CT reveals a thrombus in the right ventricle with hyper-metabolic activity (white arrow); (C), Transthoracic echocardiogram (TTE) discloses a rounded moving 30 mm long image (white arrow) sticked on the right ventricle apex with heterogeneous echogenicity, compatible with moving thrombus; (D), TTE performed 2 months later showing total resolution of ICT.