Diagnosis of Behçet ’ s Disease in Patients with Intracardiac Thrombi : A Real Big Challenge

Results: A total of 154 of BD complicated by ICT were published from 2000 to end of 2014. Sixty-seven cases were described in case series while 77 case reports were available for analysis. The most commonly reported clues and keywords that were mentioned in these reports to describe the clinical picture of patients with BD and ICT were dyspnea, fever, hemoptysis, right sided cardiac thrombi, associated pulmonary artery aneurysms, high incidence of pulmonary thromboembolic events, systemic venous occlusion, orogenital ulcers, elevated inflammatory markers, recurrence following surgical resection, and death due to fatal hemoptysis. Thirty-five percent of patients did not fulfill the international study group criteria for diagnosis of BD. Diagnosis was made based on the presence of orogenital ulcers with or without manifestations of other systems affection. About one third of the patients had undergone surgical resection of the ICT. The majority of these cases were not diagnosed as BD prior to the surgical intervention. The diagnosis of BD was made retrospectively based on the histopathological findings or following recurrence, and post-operative appearance of orogenital ulcerations.