Korean J Intern Med.  2006 Mar;21(1):50-56. 10.3904/kjim.2006.21.1.50.

A Case of Behcet's Disease with Pericarditis, Thrombotic Thrombocytopenic Purpura, Deep Vein Thrombosis and Coronary Artery Pseudo Aneurysm

• Kwon CM
• Lee SH
• Kim JH
• Lee KH
• Kim HD
• Hong YH
• Lee CK

• KMID: 1782253
• DOI: http://doi.org/10.3904/kjim.2006.21.1.50

Abstract

Behcet's disease with concomitant thrombotic thrombocytopenic purpura (TTP), coronary artery stenosis and coronary artery pseudo aneurysm is rare. Here we report a case of Behcet's disease with several cardiovascular complications, namely: pericarditis, deep vein thrombosis (DVT), TTP, coronary artery stenosis, and a coronary artery pseudo aneurysm. A 37-year-old female presented with sudden dyspnea and syncope at our emergency room and underwent pericardiectomy and pericardial window formation for the diagnosis of cardiac tamponade with acute hemorrhagic pericarditis. Thereafter, TTP and DVT complicated her illness. After confirmation of Behcet's disease on the basis of a history of recurrent oral and genital ulcers and erythema nodosum, remission was achieved after treatment with methylprednisolone pulse therapy, colchicine, catheter directed thrombolysis and thrombectomy. However, whilst maintaining anticoagulation therapy, a newly developed pericardial aneurysmal dilatation was noted on follow-up radiologic evaluation. Further evaluation revealed right coronary artery stenosis and a left coronary artery pseudo aneurysm; these additional problems were treated with the nonsurgical insertion of an endovascular graft stent . At the time of writing three months later after stent insertion, the aneurysm has continued to regress and no additional complications have intervened with combined immunosuppressive therapy.

Keyword

Behcet syndrome; Pericarditis; Hemorrhagic; Purpura; Thrombotic thrombocytopenic; Venous thrombosis; Aneurysm; False

MeSH Terms

Venous Thrombosis/diagnosis/*etiology
Purpura, Thrombotic Thrombocytopenic/diagnosis/*etiology
Pericarditis/diagnosis/*etiology
Humans
Female
Echocardiography
Coronary Vessels/*physiopathology
Coronary Stenosis/diagnosis/*etiology
Cardiac Tamponade/diagnosis/*etiology
Behcet Syndrome/*complications/diagnosis
Aneurysm, False/diagnosis/*etiology
Adult