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      Stenosis and Aneurysm of Coronary Arteries in A Patient with Behcet’s Disease

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          Abstract

          Coronary artery disease is extremely rare in patients with Behçet’s disease. We report the case of a patient with a history of Behçet’s disease who was admitted in our hospital with instable angina pectoris. The patient’s electrocardiogram was normal. Coronary angiography revealed aneurysm of the distal right coronary artery with a tight stenosis of the proximal part of the posterolateral branch. These two conditions were initially treated with immunosuppressive treatment. Three years later coronary angiography showed a total occlusion of the right coronary artery treated with medical therapy. More than fourteen cases of coronary involvement were reported in the literature but the etiopathogeny and the treatment are yet unknown

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          Most cited references 16

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          Current concepts in the etiology and treatment of Behçet disease.

          Behçet disease is an idiopathic, multisystem, chronic, and recurrent disease characterized by exacerbations alternating with phases of quiescence, episodic panuveitis, and aggressive non-granulomatous occlusive vasculitis of the arteries and veins of any size with explosive ocular inflammatory attacks that primarily affect the retinal and anterior segment vasculature of the eye. The disease is characterized by endothelial dysfunction and may produce a wide variety of symptoms. In mild cases, orogenital ulcers and skin lesions are the only findings during the entire clinical course, whereas ocular lesions that occur in more than half of the cases, frequently bilateral, can eventually lead to blindness. Pulmonary, gastrointestinal, and central nervous system involvements may sometimes be life-threatening. This review examines the epidemiology, frequency, immunology, and immunohistopathology of Behçet disease with recent theories of several agents, including phosphoantigens, superantigens, heat-shock proteins, and adenosine deaminase. Perspectives on the possible roles of new etiopathological molecules, such as nitric oxide, endothelin, and homocysteine, are presented. Ocular and systemic clinical features, diagnostic criteria, classifications, laboratory, fundus fluorescein angiography, and radiologic imaging are discussed. Differential diagnosis, disease in pregnancy and childhood, and prognosis with regard to Behçet-induced systemic and ocular complications are also evaluated. Traditional and current treatments with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil are summarized and recent insights into the pharmacology and effects of thalidomide, tacrolimus (FK-506), interferon-alpha, anti-TNF-alpha blocking monoclonal autoantibody (infliximab) and soluble TNF receptor (etanercept) are reviewed. Key clinical investigations with the status of ongoing clinical trials aimed at addressing the drug's efficacy, surgical care, and studies that have raised the possibility of new therapeutic uses are also presented. The challenges posed by the drug's teratogenicity and adverse effects are also considered, if present.
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            Atherosclerosis in Behçet's Syndrome.

            We had the impression and preliminary evidence that atherosclerosis was not much increased in Behçet's syndrome (BS). Thus, we evaluated the presence of subclinical atherosclerosis in a sizeable group of patients with BS both with major organ involvement and mucocutaneous disease along with diseased and healthy controls. We studied 239 (162 M/ 77 F; mean age: 40.7+/-7.0) patients with BS. Seventy-two (32 M/ 40 F) had only mucocutaneous and/or joint disease and 167 (130 M/ 37 F) had major organ involvement. Also 100 (24 M/ 76 F; mean age: 44.7+/-7.1) patients with rheumatoid arthritis (RA), 74 (58 M/ 16 F; mean age: 39.4+/-7.0) patients with ankylosing spondylitis (AS) and 156 (83 M/ 73 F; mean age: 39.2+/-6.6) healthy controls (HC) were studied as the control groups. We used B-mode USG to assess the frequency of plaques and intima-media thickness (IMT) in the carotid and femoral arteries. Traditional atherosclerotic risk factors were also evaluated. Men and women were analyzed separately. The frequency of plaques and the mean IMT in the carotid and femoral arteries were similar between patients with BS, AS and HC and also between the 2 subgroups of BS, among both men and women. Only men with RA were found to have significantly increased frequency of carotid artery plaques after adjustment for atherosclerotic risk factors. Increased atherosclerosis is not a prominent feature of BS, even among those patients with major organ involvement.
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              Acute myocardial infarction with normal coronary arteries in a young man with the Behcet's disease.

              Behcet's disease (BD) is an inflammatory disorder of unknown origin, which usually presents with mucocutaneous, ocular, articular, vascular, gastrointestinal and central nervous system manifestations. Although cardiac involvement is not infrequent as a manifestation of Behcet's disease, coronary arteritis is very rarely reported. We suggest that the diagnosis of coronary arteritis should be considered in patients presenting acute myocardial infarction especially in young patients as underlying cause.
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                Author and article information

                Journal
                Open Cardiovasc Med J
                TOCMJ
                The Open Cardiovascular Medicine Journal
                Bentham Open
                1874-1924
                19 December 2008
                2008
                : 2
                : 118-120
                Affiliations
                [1 ]Department of Internal Medicine, University Hospital “F. Bourguiba” Monastir (Tunisia)
                [2 ]Department of Cardiology, University Hospital “F. Bourguiba” Monastir (Tunisia)
                Author notes
                [* ]Address correspondence to this author at the Department of Internal Medicine, University Hospital F. Bourguiba, 5019, Monastir, Tunísia; Tel: 21673 447; 984 Fax: 216 73 460 737; E-mail: Sonia.Hammami@ 123456fmm.rnu.tn
                Article
                TOCMJ-2-118
                10.2174/1874192400802010118
                2627529
                19430524
                © Hammami et al.; Licensee Bentham Open.

                This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License ( http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

                Categories
                Article

                Cardiovascular Medicine

                behçet’s disease, stenosis., coronary aneurysm

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