P198 Intracardiac thrombosis and vascular involvement in Behçet's disease: two sides of the same coin?
Authors: Chadli Sarra , Khibri Hajar , Fari Safaa , Moatassim Naima , Ammouri Wafaa , Maamar Mouna , Harmouche Hicham , Adnaoui Mohammed , Mezalek Zoubida Tazi 
Published: April 2023

Background/Aims

Behçet’s disease (BD) is a relapsing vasculitis that can affect vessels of all sizes and kinds. Intracardiac thrombosis (ICT) is a rare but serious complication which often presents with vascular lesions. Yet, the link between these manifestations is not fully clarified. Our study aims to describe the profile, therapeutic management, and outcomes of BD patients with ICT. Secondly, it aims to shed some light on the relationship between ICT and vascular involvement in BD.

Methods

We retrospectively conducted a descriptive and monocentric study, including 531 Behçet’s patients admitted to our department between 2010 and 2022. All patients selected fulfilled the criteria of the International Study Group of Behçet’s Disease.

Results

Twelve patients were enrolled (2%). The sex ratio (M:F) was 9:1 and the mean age was 29 ± 6 years (20-41). ICT occurred as the revealing form of BD (50%), or after a median of 4 years since the disease onset (50%). The main symptoms were dyspnea (58%), chest pain (58%), hemoptysis (41%), and dry cough (33%). Concurrent BD flares were mainly mucocutaneous (80%). Inflammatory parameters were elevated with a median ESR of 48 mm/h and CRP of 52 mg/L. High levels of BNP and troponin were noted in 58% and 16% of the cases. Hemostasis parameters were within the normal range. On transthoracic echocardiogram, ICT was located in the right (100%), and left cavities (20%), with an extension to the inferior vena cava in half of the cases (50%). Multiple thrombi were visualized (n = 4). The associated cardiac lesions were tricuspid insufficiency (50%), pericardial effusion (41%), mitral insufficiency (16%), endomyocardial fibrosis (8%), myocarditis (8%), and ischemic coronaropathy (8%). Pulmonary hypertension (58%) and right dilatation (50%) were common. Concomitant pulmonary artery involvement was frequent (83%), consisting of thrombosis (70%) and aneurysms (50%). Six patients had a past history of lower extremity DVT, either as a single episode (n = 4) or recurrent episodes (n = 2). Vascular thrombosis was also found in the superior vena cava (n = 2), suprahepatic veins (n = 2), internal jugular vein (n = 1), pulmonary veins (n = 1), cerebral vein (n = 1), superior mesenteric vein (n = 1), and femoral artery (n = 1). Patients were treated with colchicine (100%), glucocorticoids (100%), and immunosuppressants (92%), mainly cyclophosphamide (n = 11), and a TNF-alpha inhibitor (n = 2). Six patients were under curative anticoagulation. A good response to therapy was observed in all patients. One case of relapse was reported. No deaths were recorded.

Conclusion

ICT usually occurs during the first years of BD onset in young male patients presenting with vascular lesions, especially of the pulmonary arteries and vena cava. We thus believe that, unlike common thrombotic diseases, ICT in BD is a direct consequence of the underlying vasculitis. The use of immunosuppressants, with or without anticoagulants, is generally associated with a favorable outcome.

Disclosure

S. Chadli: None. H. Khibri: None. S. Fari: None. N. Moatassim: None. W. Ammouri: None. M. Maamar: None. H. Harmouche: None. M. Adnaoui: None. Z. Tazi Mezalek: None.