POS1335 CLINICAL CHARACTERISTICS OF THE PULMONARY ARTERY INVOLVEMENT IN PATIENTS WITH BEHÇET’S SYNDROME: A SINGLE-CENTRE EXPERIENCE OF 61 PATIENTS

Abstract

Background:Behçet’s Syndrome (BS) is a vasculitis of unknown origin. Vascular involvement, so-called vascular Behçet’s syndrome (VBS), may involve blood vessels of all sizes belonging to both venous and arterial system, with pulmonary artery involvement (PAI) being the most frequent form of arterial involvement. PAI in BS occurs in the form of aneurysms or thrombosis, and results in significant mortality.Objectives:To report the clinical characteristics of PAI in patients with BS and to define the predictors of relapses.Methods:We performed a retrospective analysis of BS patients with PAI who fulfilled international study group criteria. Among 460 patients with VBS, 66 were diagnosed with PAI. For final analyses, 61 patients with PAI, who had at least 2 follow-up visits (72.1% male, mean age at BS diagnosis 29.34 (SD 10.1) years), were included. The data of the patients were recorded. Relapse was defined as the reoccurrence of vascular event at any vascular structure. Factors associated with relapse were assessed by logistic regression analysis.Results:There were not any differences considering demographic and clinical features of the patients with and without PAI in VBS group except for that intracardiac thrombosis was more common in the patients with PAI than the patients without (19.7% vs 0.3%, respectively) (Figure 1). Among 61 patients with PAI, 50 (82.0%) had isolated pulmonary artery thrombosis (PAT), whereas 11 (18.0%) had pulmonary artery aneurysm (PAA) with or without PAT. The characteristics of 61 patients with PAI were shown in Table 1. A total of 37 relapses occurred in 24 (39.3%) patients during follow-up a median of 65.9 (IQR 20.1-109.0) months. To define the factors associated with relapses, the patients with isolated PAT were analysed. In multivariable logistic regression analysis, older age at BS diagnosis and anticoagulation usage seemed to be protective, even though they could not reach statistical significance (OR: 0.92 95% CI 0.86-1.02, OR: 0.34 95% CI 0.09-1.33, respectively).Table 1.Characteristics of the patients with pulmonary artery involvementAlln= 61Isolated PATn= 50PAAn= 11Sex (male), n (%)44 (72.1)35 (70.0)9 (81.8)Age at diagnosis of BS, years (SD)29.3 (10.1)28.8 (10.4)31.9 (8.0)Age at diagnosis of PAI, years (SD)36.3 (12.83)36.7 (13.8)34.7 (6.7)Symptoms, n (%)Dyspnea28 (45.9)23 (46.0)5 (45.5)Hemoptysis26 (42.6)19 (38.0)7 (63.6)Vascular event before PAI, n (%)21 (34.4)20 (40.0)1 (9.1)Other vascular events at PAI, n (%)33 (54.1)28 (56.0)5 (45.5)Thrombophlebitis, n (%)7 (21.2)6 (21.4)1 (20.0)Upper and/or lower extremity DVT, n (%)20 (60.6)16 (57.1)4 (80.0)SVCS, n (%)1 (3.0)1 (3.6)0 (0)IVCS, n (%)3 (9.1)1 (3.6)2 (40.0)Intracranial thrombosis, n (%)4 (12.1)4 (14.3)0 (0)Intracardiac thrombosis, n (%)8 (24.2)7 (25.0)1 (20.0)Arterial event other than PAI, n (%)4 (12.1)3 (10.7)1 (20.0)Relapse, n (%)24 (39.3)20 (40.0)4 (36.4)Time to the first relapse, median (IQR), month49.8 (13.2-102.6)54.1 (24.4-117.9)3.9 (1.7-67.9)Death, n (%)2 (3.3)0 (0)2 (18.2)BS= Behçet Syndrome, PAI= pulmonary artery involvement, PAT= pulmonary artery thrombosis, PAA= pulmonary artery aneurysm, DVT= deep vein thrombosis, SVCS= superior vena cava syndrome, IVCS= inferior vena cava syndromeFigure 1.A) Axial CT image shows thrombus formation in the left upper lobe pulmonary artery branch. B) Giant thrombus is seen in the right ventricle (black arrow). Note the thrombus formation in the left lower lobe pulmonary artery branches (white arrows).Conclusion:Our results indicate that there is a higher frequency of intracardiac thrombosis in BS patients with PAI and an increasing current trend of PAT in patients with PAI. In addition, there might be a possible efficacy of anticoagulation usage in preventing relapses, which needs confirmation with further studies.Disclosure of Interests:None declared