Importance of Echocardiographic Findings in the Acute Presentation of Behçet's Disease – Diagnostic and Prognostic Considerations

This issue of the Journal contains 2 interesting papers from the Mediterranean region (Egypt and Israel), describing intracardiac thrombi as the initial cardiac presentation of Behçet’s disease (BD). Farouk et al. present a large series of patients with BD observed in their institution. These patients were separated into 2 clinical subsets: 41 patients treated chronically with an established diagnosis of BD and 5 patients admitted with acute BD manifestations. The authors noted striking differences in the frequency of positive echocardiographic findings among these 2 clinical groups. All patients with acute onset BD had positive echocardiographic findings. The authors noted a very high incidence of intracardiac thrombosis (4 of 5 patients) in the group with acute BD presentation. In all cases, the thrombi were found in the right heart (3 patients had right atrial thrombus and 1 patient had thrombi seen both in the right atrium and the right ventricle). One additional patient in this group had a pericardial effusion. Contrary to the patients with acute onset BD, there were no intracardiac thrombi found in patients with chronic stable BD. Only one of the 41 patients in this group had positive echocardiographic findings (severe aortic regurgitation and a significantly dilated left ventricle). Leibowitz et al. describe 2 clinical cases of acute inflammatory BD with a right ventricular mass. In both cases (35-year-old man and 9-yearold boy), the RV mass was considered a significant finding requiring surgery. The patients then underwent resection of the RV mass. According to the authors’ report, in both cases, histopathology of the mass was consistent with thrombus. Of note, there was a recurrence of the thrombus following surgery, and a final resolution of the problem was achieved only after initiation of aggressive anti-inflammatory therapy. These studies demonstrate the importance of considering BD in the differential diagnosis of patients with an acute presentation suggesting an inflammatory etiology (fever, high erythrocyte sedimentation rate, fatigue) and intracardiac mass. The probability of right heart thrombus appears to be very high in patients presenting with the acute symptoms as well as hemoptysis and cough. In this regard, as Farouk et al. state, “. routine inquiry about the occurrence of oral/genital ulcers can be of great value in the early diagnosis and treatment of BD with subsequent prevention of the development of (intracardiac thrombus) among these patients. Adding routinely the question about orogenital ulcers to the medical records of adolescents and young males seems simple, easy and of great cost-benefit value. ” We definitely support this important statement. Based on these papers as well as analysis of previously published data, careful and complete echocardiographic examination in patients with acute BD presentation becomes a definite clinical necessity. BD is typically defined as a systemic inflammatory condition with the following clinical features: (1) orogenital ulcers (aphthae or herpetiform lesions – main diagnostic criterion); (2) ocular involvement (uveitis or retinal vasculitis); (3) cutaneous findings (erythema nodosum, acneiform nodules, papulopustular lesions, or pseudofolliculitis); (4) positive pathergy test. Address for correspondence and reprint requests: Aiden Abidov, M.D., Ph.D., Division of Cardiology, The UAMC Cardiovascular Imaging, The University of Arizona, 1501 N Campbell Avenue, Tucson, AZ 85724. Fax: 520-626-4333; E-mail: aabidov@shc.arizona. edu