Behçet's syndrome: pulmonary vascular disease

Pulmonary artery involvement (PAI) due to Behçet's syndrome, although rare, has always been a focus of interest not only in endemic areas but also in many parts of the world. This interest is shared by many disciplines other than internal medicine, such as radiology, cardiothoracic surgery, cardiology and respiratory medicine. Most importantly, our understanding of PAI has significantly changed in recent years.

Recent work found the following: pulmonary artery aneurysms are not the only form of PAI. Solo 'in-situ' pulmonary artery thrombosis (PAT) can be found in up to one-third of the patients at presentation. Both pulmonary artery aneurysms and PAT present with similar clinical symptoms, whereas abundant hemoptysis is less frequent in PAT. PAI is strongly associated with thrombosis in the lower-extremity deep veins and right side of the heart. Varying and multiple pulmonary parenchymal lesions such as nodules, consolidations and cavities are part of this involvement as well. While either aneurysms or thrombosis could disappear successfully with immunosuppressives in up to 70% of the patients, the condition can be fatal still in one-fourth. Large aneurysms and high pulmonary artery pressures are associated with a poor prognosis.

Although we now know the condition and its associated complications better, we need to study how we can predict development of PAI, its pathogenesis and finally what can we do to improve the survival.