Cardiac Involvement in Behçet's Disease

Mechanisms of Cardiac Involvement

The American Heart Association acknowledges that Behçet's disease is a small vessel vasculitis that uniquely affects both arteries and veins of all sizes, making it one of only two vasculitides with venous involvement, with vasculitic inflammation and destruction of the arterial media leading to aneurysm formation, pseudoaneurysm development, and potential rupture 1
The pathophysiology of Behçet's disease involves granuloma formation in the adventitial layer with augmented cytokine and matrix metalloproteinase production causing vessel destruction, according to the European League Against Rheumatism 3, 2

Aneurysm formation can occur at multiple sites and progress unpredictably to rupture, which can be fatal, with anastomotic pseudoaneurysms occurring in 12.9% of patients within 18 months after surgical repair due to ongoing inflammation at suture lines, as reported by the American College of Cardiology 1
The ascending aorta and aortic root can be affected, though less commonly than in other vasculitides, with deep vein thrombosis and superficial thrombophlebitis being common manifestations, according to the American Heart Association 1, 3

Young men with early disease onset face the highest risk of severe cardiac and vascular complications, requiring aggressive early immunosuppression, with male gender and early disease onset being high-risk features for cardiac involvement, as recommended by the European League Against Rheumatism 4, 6

For acute cardiac involvement, treatment follows the same immunosuppressive principles as other major organ involvement, with high-dose glucocorticoids, azathioprine, and cyclophosphamide being used, as suggested by the American College of Rheumatology 4
Anticoagulants should generally be avoided in Behçet's disease with venous thrombosis due to bleeding risk, especially with coexisting pulmonary arterial aneurysms, with immunosuppressive agents being the primary treatment, according to the American Heart Association 4, 5

TNF-α inhibitors show promise, particularly when combined with azathioprine, for severe or refractory cardiac involvement, with screening for tuberculosis being necessary before initiating anti-TNF therapy, as recommended by the European League Against Rheumatism 4