To determine the frequency of elevated systolic pulmonary artery pressure (sPAP) estimated by echocardiography in Behçet’s syndrome (BS) patients with pulmonary artery involvement (PAI), in healthy controls and in diseased controls with systemic sclerosis (SSc), as well as in BS patients without PAI.

We studied 3 groups of patients with BS (patients with PAI: n = 30, with vascular disease but without PAI: n = 26 and without vascular disease: n = 21), patients with SSc (n = 23) and healthy controls (n = 22). Systolic pulmonary artery pressure (sPAP) was estimated by echocardiography. The upper limit for a normal sPAP was arbitrarily set at 35 mmHg. We also evaluated cardiac function by echocardiography. Pulmonary function tests, a six-minute walking test (six-MWT) and several serum biomarkers were also studied.

The frequency of patients with an elevated sPAP was significantly higher only among BS patients with PAI (17%) and among patients with SSc (26%). In addition, DL_CO was decreased and pro-BNP levels were increased in BS patients with PAI, which are similar to the results in patients with SSc. Furthermore, BS patients with PAI also had mild RV diastolic dysfunction.

When BS involves the pulmonary arteries, it can cause mild elevations in the estimated sPAP, decrease in DL_CO, mild cardiac dysfunction and increase in pro-BNP levels. These findings suggest that BS can also affect the small/micro vessels of the heart and the lungs in addition to the well-recognized large vessel disease.