Pulmonary artery thrombosis in Behcet's syndrome: a case series

Abstract

Abstract Background Behçet's syndrome (BS) is a complex multisystemic vasculitis that affects vessels of all sizes and kinds, with a high tendency towards thrombosis. Arterial disease is rare and manifests primarily in the form of pulmonary artery aneurysms (PAA), but also of pulmonary artery thrombosis (PAT) in 1/3 of the cases. Purpose Our work aims to depict the profile and outcomes of BS patients presenting with PAT. Methods This retrospective, descriptive, and monocentric study was conducted between 2000 and 2022, among 531 adult patients diagnosed with BS (ISG and/or ICBD criteria). All cases of documented PAT were included. Results 16 cases of PAT were recorded (3%). The mean age of the patients was 34 ± 6 years (19-58), and the sex ratio (M/F) was 4.7:1. Cardiovascular risk factors were mainly active smoking (12%) and high blood pressure (6%). PAT was the revealing manifestation of BS (30%), or occurred after a median of 4.5 years following BS onset. Patients were mostly asymptomatic (69%), or presented with dyspnea (35%), hemoptysis (31%), and thoracic pain (25%). On chest CTA, PAT was often bilateral (78%) and proximal (46%). PAA were frequently observed (25%), being consistently bilateral, multiple, proximal and distal at the same time. Deep venous thrombosis was found in the lower extremities (19%), superior vena cava (27%), and inferior vena cava (6%). Cardiac involvement was also commonly associated (40%), mostly consisting of right intracardiac thrombosis (37%). Concomitant extracardiovascular involvements included mucocutaneous (87%), ocular (26%), articular (25%), and digestive (6%) lesions. Inflammatory parameters were raised in most patients (80%). Hemostasis parameters were within normal ranges and screening for inherited and acquired thrombophilia was negative in all cases. Patients were treated with colchicine (97%), glucocorticoids (93%), cyclophosphamide (47%), azathioprine (47%), and a TNF-a-inhibitor (6%). Curative anticoagulation with low-molecular-weight heparin followed by oral antivitamin K was often prescribed (60%). No surgery or endovascular procedure was performed. Relapse and death were recorded in respectively 15% and 26% of the cases. Conclusion In contrast to common thrombotic diseases, PAT in BS is attributed to vasculitis rather than hypercoagulability, resulting in distinct clinical features. The diagnosis should be promptly considered in young men who present with concurrent thrombotic lesions and PAA. Early and appropriate management is imperative to enhance patient outcomes.Left proximal PAT in a BS patient