A 65-year-old man presented with chest discomfort. Contrast-enhanced computed tomography revealed a large mass shadow (34 mm) at the right atrioventricular groove (Figure A), which was later confirmed to be a giant thrombosed aneurysm at the ostium of the right coronary artery by magnetic resonance imaging (Figure B). Large saccular descending aortic and abdominal aortic aneurysms were also detected (Figure C–E) and the patient underwent surgical graft replacement.
The patient’s medical history included a diagnosis of Behcet’s syndrome at age 20 years, followed by long-term glucocorticoid therapy. Adjunctive methotrexate therapy was occasionally introduced for the control of inflammation. At age 43 years, he received Y-graft surgery for an abdominal aortic aneurysm. At age 59 years, he developed an acute inferior myocardial infarction (Figure F) with multivessel coronary artery disease (Figure G,H), and underwent coronary artery bypass grafting (Figure I).
Coronary arteries are rarely affected,1,2 and a giant coronary artery aneurysm coupled with recurring aortic aneurysms at multiple locations is most exceptional. The patient had no history of Kawasaki disease, and immunoglobulin G4-related disease was thought unlikely based on the histopathological features of the aorta. Observation spanning more than 45 years in this patient may shed light on the lifetime clinical course of vascular Behcet’s syndrome.
References
- 1.
Saadoun D, Bodaghi B, Cacoub P. Behcet’s syndrome. N Engl J Med 2024; 390: 640–651.
- 2.
Demirelli S, Degirmenci H, Inci S, Arisoy A. Cardiac manifestations in Behcet’s disease. Intractable Rare Dis Res 2015; 4: 70–75.
