Coronary atherosclerosis in Behçet's syndrome: a pilot study using electron-beam computed tomography

E. Seyahi, E. Memisoglu1, V. Hamuryudan, S. Tepe2, U. T. Aker3, H. Balci, Z. Ongen, S. Yurdakul and H. Yazici

Rheumatology, Cerrahpasa Medical Faculty, Istanbul, Turkey, 1 Radiology, Saint Louis University, St Louis, MO, USA, 2 TEST Cardiovascular Imaging, Nisantasi, Sezai Selek sok No. 21, Istanbul, 3 Kardio-tek Center, Valikonagi cad. No. 173 D:7/4, Nisantasi, Istanbul, Turkey

Correspondence to: V. Hamuryudan, Rheumatology Department, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey. E-mail: vedath{at}isbank.net.tr  E. Seyahi and E. Memisoglu contributed equally to this work.

SIR, Behçet's syndrome (BS) may involve vessels of virtually all types and sizes but coronary artery disease has been reported sporadically. Coronary atherosclerosis in BS has not formally been studied thus far.

Electron beam computed tomography (EBCT) has emerged as a simple and non-invasive technique for the diagnosis of coronary atherosclerosis [1, 2]. It detects coronary artery calcification, which is a potential indicator of significant future coronary risk and also shows the wall morphology and patency of coronary arteries with contrast enhancement [1–3].

We investigated the frequency of coronary atherosclerosis, by using EBCT, in a group of male BS patients selected specifically for having major vessel disease and long disease duration. Our hypothesis was that coronary atherosclerosis would be frequent in this group of BS patients. We defined major vascular involvement as the presence of an aneurysm and/or arterial occlusion and/or thrombosis of the venae cavae. All patients were initially screened for cardiovascular risk factors. Total and individual coronary calcium scores were calculated according to the method described by Agatston et al. [3]. Coronary artery calcium scores were stratified as follows: (i) no calcification; (ii) 1–100, minimal to slight calcification; (iii) 101–400, intermediate calcification; (iv) score greater than 400, extensive calcification. The study was approved by the local ethics committee of Cerrahpasa Medical Faculty and informed consent was obtained from all patients.

We studied 24 patients [mean age 37.8 ± 4.5 (S.D.) yr; duration of vascular involvement 10.1 ± 3.7 yr]. The main vessels involved were the pulmonary arteries (nine patients), abdominal aorta (four patients) and vena cava (nine patients). Seven patients had more than one diseased vessel. Fifteen patients (63%) had used corticosteroids previously, with a mean duration of 3.9 ± 3 yr. All patients were previous or current users of cyclophosphamide or azathioprine. The mean duration of treatment with these drugs was 4.3 ± 3 yr. The majority (88%) smoked and 42% had at least mild lipid abnormalities. One patient had diabetes mellitus. No patient had arterial hypertension or myocardial infarction. Coronary artery calcium scores were zero in 21 (88%) patients. Two patients had scores suggesting minimal or slight calcification (58 and 7) and one patient had a calcium score of 600, indicating extensive calcification. These three patients (12%) were also the only ones with abnormal imaging on EBCT coronary angiography, as shown in Fig. 1. One patient had total occlusion and calcified aneurysm in the proximal part of the left anterior descending artery (LAD) (Fig. 1, left panel). The second had mild stenosis with calcified plaque formations in the LAD (Fig. 1, middle panel), and the third had non-calcified aneurysm in the LAD (Fig. 1, right panel). Our results suggest that coronary artery abnormalities are not common in BS even in a selected group of male patients.



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FIG. 1. Cranial right anterior oblique (RAO) views: volume-rendered three-dimensional EBCT angiography of the heart after segmentation of the ascending aorta and pulmonary artery. (A) 39-yr-old male patient with vena cava thrombosis and chest pain. CAC score 600. There is proximal occlusion of the LAD with a calcified, thrombosed mid-LAD aneurysm (arrow). Flow in distal LAD is retrograde via collaterals. Thallium scintigraphy was consistent with anterobasal ischaemia. Conventional coronary angiography was also in accord with the finding of EBCT. Although severe, the occlusion in the proximal part of the LAD was too short and narrow for a surgical intervention so medical treatment was carried out. (B) 40-yr-old male patient with pulmonary artery aneurysm and inferior vena cava occlusion. CAC score 58. There is intermediate stenosis of the mid-LAD (arrowhead). Calcified plaques are also noted in the proximal LAD and 2nd diagonal branch. (C) 36-yr-old male patient with pulmonary artery aneurysm and inferior vena cava occlusion. CAC score 7. Non-calcified proximal LAD aneurysm (open arrow). No further investigation was done in the last two patients as their scintigraphy was normal. RVOT, right ventricular outflow tract; LA, left atrium; RA, right atrium; Ao, aorta; PA, pulmonary artery; RCA, right coronary artery; CCA, circumflex coronary artery.

 
In a recent study using EBCT, coronary artery calcification (CAC) was described among 31% of 65 SLE patients with no history of coronary artery disease, compared with 9% of 69 controls. The patients and controls in this study were predominantly women and had an average age of approximately 40 yr [4]. The prevalence of CAC among women is estimated to be half that in men until the age of 60 yr [1]. Extrapolating from this, and not forgetting the limitations of indirect comparisons, especially among different ethnical groups, the frequency of CAC among our selected male patient population seems to be at least not increased when compared with what is found in the general population. Unfortunately, data on coronary calcification in the general population of Turkey are not available and our study did not include a formal control group.

Among 350 patients with BS who were followed up between 1974 and 1993 in France, three (0.09%) had coronary arterial involvement [5]. Furthermore, in a 20-yr outcome survey on 387 patients from our centre, only three of the 42 deaths (0.7%) were due to coronary artery disease [6]. In that study the mortality in BS was mainly due to extracardiac vascular or neurological disease, and these complications showed a tendency to decrease in frequency with the passage of time during the disease course. This mortality pattern is different from what is found in RA and SLE [7, 8], in which there is an increased or bimodal mortality pattern with increased atherosclerotic coronary artery disease with the prolonged disease course.

It is believed that the classical risk factors, such as smoking and high lipid levels, are important in the coronary atherosclerosis of inflammatory diseases. Disease duration, corticosteroid use and inflammatory activity are implicated as additional risk factors [9]. In our study, coronary atherosclerosis was lower than what we expected, although our patients carried most of these risk factors. The relatively young age of our patients or the immunosuppressive treatment employed might have played a role in this outcome. Another explanation could be that the disease activity in BS is more short-lasting compared with other diseases characterized by chronic and long-lasting inflammation, such as SLE and RA [6].

In summary, our preliminary findings indicate a relatively low frequency of coronary atherosclerosis in BS even when studied under worst-case conditions. Further studies are needed, first to verify these preliminary observations in a controlled setting and secondly to elucidate their biological importance, if verified.

This study was supported by the Research Fund of the Istanbul University (Project No: 1529/16012001).

The authors have declared no conflicts of interest.

References

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Accepted 9 July 2004





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