Background Behçet’s disease (BD) is a systemic vasculitis that involvement of pulmonary arteries can be seen.

Objectives The aim of this study was to determine the frequency and the causes of pulmonary hypertension (PH) in patients with BD.

Methods We studied consecutively 154 BD patients who were fulfilled the International Study Group criteria for diagnosis of BD. All patients were evaluated with transthoracic echocardiography (TTE) for the presence of PH. BD patients were categorised according to the involved organs in 5 groups: group 1 mucocutaneus and articular, group 2 ocular, group 3 vascular, group 4 gastrointestinal and group 5 neurologic involvements. The presence of PH was defined as estimated sPAB ≥40 mmHg, by TTE. Every subject evaluated by a detailed medical history and physical examination was performed. Additional laboratory results were obtained from hospital file records.

Results The mean age (SD) and the median (min-max) disease duration of the patients were 41.8±12.6 years and 126 (6–540) months respectively. PH was detected in 17 (11%) BD patients. Only 9 (52%) patients were symptomatic (NYHA FC >1). Left sided heart disease (Group II: 9 (52%) patients) was the leading cause of PH. Four (23%) patients had group IV PH and 75% (3/4) were symptomatic. Diastolic dysfunction (DD) was found in 32 (20.8%) patients and only 1 patient had systolic dysfunction. The number of patients with DD was significantly higher in patients with PH as compare to patients without PH (8 (47.1%) vs 24 (17.6%), p=0.005). There were no difference in demographic and clinical features of patients with and without PH. Only acneiform lesion was more frequently in patients without PH as compare to patients with PH, p=0.047 (table 1). There were no differences in frequency of PH in BD groups (table 2).

Conclusions The most frequent cause of PH in BD patients was group II PH. There were also significant number of patients with group IV PH. Pulmonary endarterectomy may be an option of treatment in inactive patients with group IV PH.

Disclosure of Interest None declared