POS0792 PULMONARY ARTERY INVOLVEMENT IN BEHCET’S DISEASE: SINGLE CENTER RESULTS

Abstract

BackgroundBehcet’s disease (BD) is a multisystem inflammatory vasculitis. Skin, mucosa, eye, vascular area, joint, gastrointestinal system and central nervous system involvement is observed.ObjectivesIn this study, we aimed to present the data of patients with pulmonary artery involvement (PAI) followed up with the diagnosis of BD.MethodsThe clinical, demographic and laboratory data of 394 patients with the diagnosis of BD, who were followed up in our rheumatology outpatient clinic between 2000 and 2020, were evaluated retrospectively.ResultsOf the patients followed up with the diagnosis of BD, 44% were female and 56% were male. Oral aphthous ulcers were found in 96%, genital ulcers in 65%, papulopustular lesions in 33%, erythema nodosum in 38%, and pathergy positivity in 47% of the patients.PAI was detected in 3% (n:13) of the patients who were followed up with a diagnosis of BD. 69% of the patients who had PAI were male, and their mean diagnosis age was 27±9, disease duration was 10±4.7 years. Pulmonary artery aneurysm was observed in 62%, pulmonary artery thrombosis (PAT) was observed in 85%, and both conditions were observed in 46% of the patients. Oral aphthous ulcer were found in all patients, genital ulcers in 70%, papulopustular lesions in 23%, pathergy positivity in 39%, and erythema nodosum in 23%. One of the patients with PAI had hereditary thrombophilia and also lower extremity deep vein thrombosis (DVT). Cardiac involvement was in the form of intracardiac thrombus in patients with PAI.Patients with and without PAI were compared in terms of clinical findings. A significant difference was observed in terms of DVT in the lower extremity, venous and cardiac involvement. The relationship between PAI and these involvements was also shown in the regression analysis (Table 1).Table 1.Comparison of data of patients with and without pulmonary artery involvementN (%)Pulmonary Artery Involvement (+)Pulmonary Artery Involvement (-)POR (%95 CI)*N=13N=381Gender Female4(30)168(44)0.341 Male13(70)213(56)Family History2(15)46(12)0.664Oral Aphthous Ulcer13(100)366(96)1Genital Ulcer9(70)247(65)1Papulopustular lesion3(23)126(33)0.559Erythema Nodosum3(23)147(39)0.385Pathergy positivity5(39)180(47)0.738Uveitis-138(36)NARetinal Vasculitis-11(3)NAArthritis1(8)97(26)0.200Venous Involvement8(62)68(18)0.0017.365(2.33-23.20)Lower Extremity Deep Venous Thrombosis6(46)46(12)0.0036.24(2.01-19.38)Peripheral Artery Aneurysm-5(1)NAGIS involvement-17(5)NACardiac involvement3(23)4(1)0.00138.27(5.57-143.25)Cranial Involvement-24(6)NA*Significant data in logistic regression analysis were presented, GIS: Gastrointestinal systemCranial involvement was not detected in any of our patients with PAI. In subgroup analysis, a significant relationship was observed between PAT and intracardiac thrombus (p=0.001 OR:21.05 95%CI 3.4-130) and lower extremity DVT (p=0.001 OR: 8.79 95%CI 2.58-29.96).ConclusionPAI is rare but the most important involvement affecting mortality in BD patients. Recently, there has been an increase in the incidence of isolated PAT with the contribution of the developments in imaging methods.1 PAI is associated with lower extremity DVT, cerebral venous thrombosis and intracardiac thrombus.2 In our study, a significant correlation was found between PAI and the presence of venous involvement, lower extremity DVT and intracardiac thrombus.