title: A case of Behçet's disease with pericarditis, thrombotic thrombocytopenic purpura, deep vein thrombosis and coronary artery pseudo aneurysm.
abstract: Behçet's disease with concomitant thrombotic thrombocytopenic purpura (TTP), coronary artery stenosis and coronary artery pseudo aneurysm is rare. Here we report a case of Behçet'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 Behçet'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.
authors.affiliation: Division of Rheumatology, Department of Internal Medicine, Yeungnam University College of Medicine, Daegu, Korea.
authors.forename: Chang Mo
authors.initials: CM
authors.lastname: Kwon
authors.forename: Seung Hyun
authors.initials: SH
authors.lastname: Lee
authors.forename: Jin Ho
authors.initials: JH
authors.lastname: Kim
authors.forename: Kyu Hyung
authors.initials: KH
authors.lastname: Lee
authors.forename: Hyun Do
authors.initials: HD
authors.lastname: Kim
authors.forename: Yeong Hoon
authors.initials: YH
authors.lastname: Hong
authors.forename: Choong Ki
authors.initials: CK
authors.lastname: Lee
mesh_terms: D000328:Adult; D017541:Aneurysm, False / Q000175:diagnosis / Q000209:etiology*; D001528:Behcet Syndrome / Q000150:complications* / Q000175:diagnosis; D002305:Cardiac Tamponade / Q000175:diagnosis / Q000209:etiology*; D023921:Coronary Stenosis / Q000175:diagnosis / Q000209:etiology*; D003331:Coronary Vessels / Q000503:physiopathology*; D004452:Echocardiography; D005260:Female; D006801:Humans; D010493:Pericarditis / Q000175:diagnosis / Q000209:etiology*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology*; D020246:Venous Thrombosis / Q000175:diagnosis / Q000209:etiology*