Cardiovascular involvement in Behçet’s disease [Fig.
• Most common venous manifestation such as superficial thrombophlebitis
– Aneurysm ; thoracoabdominal aorta and branch vessels
– Occlusion ; less frequent than aneurysm,
– Pseudoaneurysms - on the aortic root,
aneurysm of coronary artery : extremely rare
– Venous thrombosis - deep and superficial vein and vena cava
• Cardiac involvement: 7~46%,
The incidence and nature is not yet clearly documented
• Aneurysm of sinus of Valsalva,
aortic or mitral valve involvement,
proximal aortic dilatation,
thrombus on right atrium (RA),
interatrial septal aneurysm and myocardial infarction from coronary arterial occlusion
Coronary involvement of IgG4 related vasculitis [Fig.
• IgG4-related disease has not been well documented in the cardiovascular system.
• lSome patients with inflammatory abdominal aorta aneurysm (AAA) had high serum IgG4 concentrations and diffuse lymphoplasmacytic infiltration including numerous IgG4-positive plasma cells.
• IgG4-related inflammatory AAA could be estimated as IgG4-related periaortitis together with retroperitoneal fibrosis.
• IgG4-related periarteritis could occur in the cardiovascular system and might manifest aneurysm or a tumorous lesion along the artery.
Coronary and cardiac involvement in Churg-Stauss syndrome [Fig.
• Acute and constrictive pericarditis,
and ischemic cardiomyopathy / Pericardial effusion and myocardial dysfunction
• Accounts for 50% of deaths in Churg-Strauss syndrome
• MR Finding
– Typical for delayed wash-out of contrast medium due to interstitial fibrosis with increased extracellular space.
but regional limited findings for subendocardial fibrosis following myocardial infarction
Coronary involvement in Moyamoya disease [Fig.
• Progressive narrowing of distal ICA and proximal circle of Willis vessels with secondary collateralization
• Reported involvement of other location ; Renal artery,
and coronary artery