An unusual case of chronic and reversable pulmonary hypertension

Behcet’s Disease (BD) is currently classified as “variable vessel vasculitis” (Chapel Hill, 2012). It is a chronic, multisystemic, inflammatory rheumatic disease. Its main manifestations are mucocutaneous, neurological, cardiovascular, pulmonary and musculoskeletal. Vascular involvement of BD, so-called “angio-Behcet”, appears in 7%-29% of patients. Arterial complications, such as arterial stenoses, pseudoaneurysms, and ruptures occur in a 1-14% range and represent a major cause of morbidity and mortality in BD patients. We, herein, report the case of a large aortic pseudoaneurysm producing severe pulmonary hypertension in a patient with angio-Behcet who had previously undergone aortic root replacement.