Abstract
Behçet disease (BD) is a multisystem disorder, with vasculitis as its underlying pathological
process, in contrast to the classic triad of recurrent oral and genital ulcerations,
with uveitis. Vascular involvement in BD includes venous thrombosis, arterial occlusion,
and pulmonary artery and aortic aneurysm formation. Cardiac involvement is rare and
often obscure. It includes intracardiac thrombi formation, and is associated with
a poor prognosis. Our objectives are to describe two cases with BD, complicated with
vascular and cardiac involvement, and to raise awareness of these rare complications,
the needed routine surveillance, and thus to prevent inappropriate interventions,
serious outcomes, and mortality. We present two male patients from the Mediterranean
Basin with BD. The first was diagnosed early as a BD patient. The second was diagnosed
at the time of cardiovascular (CV) involvement. We recommend that patients who are
diagnosed, or even suspected of suffering from BD, especially in endemic areas along
the Silk Route pathway, should be followed up routinely for CV involvement, even if
rare, obscure, or with a bizarre presentation.
Keywords
Behçet disease - cardiovascular involvement - vasculitis - pulmonary embolism - intracardiac
thrombus
