POS1345 BEYOND PULMONARY ARTERY ANEURYSM; PULMONARY INVOLVEMENT IN BEHCET’S SYNDROME

Abstract

BackgroundPulmonary involvement (PI) other than pulmonary artery aneurysm (PAA) in Behcet’s syndrome (BS) is still an area of investigation.ObjectivesHerein, we aimed to retrospectively evaluate all types of pulmonary involvement associated with Behcet’s syndrome (BS).MethodsAmong 800 BS patients according to International Study Group for Behcet’s Disease (ISG) criterias, 28 patients were selected based on their radiologic examination consistent with BS-related PI. Demographic features, other clinical manifestations of BS, treatment modalities and types of PI were analyzed.ResultsThe overall prevalence of PI was estimated 3,5% among all BS patients. PI was more common in males (82.1% vs 17.9%). Mean age for BS diagnosis and onset of PI were as following; 32 ± 10,9 and 37 ± 11,4 years. Deep vein thrombosis (DVT) was the most common accompanying vascular involvement (53,6%). PAA, pulmonary vasculitis (PV), and pulmonary thromboembolism (PTE) were seen in 7 (25%), 13 (46,3%), and 18 (64,4%) of patients, respectively. In 5 patients, intracardiac thrombosis was present simultaneously in the right ventricle. Cyclophosphamide (CYC) was the most common preferred agent (78%) followed by azathioprine (AZA) as first line. Warfarin used in 18 patients (Table 1) Mortality was seen in 3 patients during follow up (1 due to PAA bleeding, others with unknown causes).Table 1.Characteristics of pulmonary involvement in BS patientsMale/Female, (n), (%)23/5 (82,1%/17,9%)Age of diagnosis, years (Mean± SD)32 ± 10,9Age of pulmonary involvement, years (Mean ± SD)37 ± 11,4Oral ulcer, (n), (%)28 (100%)Genital ulcer, (n), (%)20 (71,4%)Osteofollicular lesion,(n), (%)20 (71,4%)Erythema nodosum, (n), (%)13 (46,4%)Uveitis, (n), (%)8 (28,6%)DVT, (n), (%)15 (53,6%)Cardiac involvement, (n), (%)5 (17,8%)Pulmonary involvement, (n)-PAA, (n), (%)287 (25%)-PV, (n), (%)13 (46,4%)-PTE, (n), (%)18 (64,3%)Immunosuppression (first line agents)26/28-Steroid, (n), (%)26/28-CYC, (n), (%)22/26 (84,6%)-AZA, (n), (%)4/26 (15%)Anticoagulation (warfarin), (n), (%)18PAA; pulmonary artery aneurysm, PTE; pulmonary thromboembolism, PV; pulmonary vasculitis, CYC; cyclophosphamide, AZA; azathioprine, DVT; deep vein thrombosisConclusionDespite the importance of PAA in BS patients, capillaritis and thromboembolism other than PAA may occur commonly in BS as well. Onset at young age, male gender and previous DVT seem to be significant risk factors for the development of PI.