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      Superior vena cava thrombosis and dilated cardiomyopathy as initial presentations of Behcet’s disease

      case-report

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          Abstract

          Background

          Bechet’s disease (BD) is a relatively rare disease that causes recurrent oral and genital ulcers in addition to a variety of systemic manifestations. Concomitant superior-vena-cava (SVC) thrombosis and cardiac involvement with dilated cardiomyopathy (DCM) as initial presentations for BD is considered rare.

          Case presentation

          A 32-year-old-man presenting with intractable headaches and dyspnea. He was later diagnosed with SVC thrombosis and DCM. A diagnosis of BD was made after detailed history-taking.

          Conclusions

          Cardiovascular manifisations can be the initial presentation of BD. We aim to highlight the importance of early clinical recognition of BD as a cause of DCM and SVC thrombosis.

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          Most cited references18

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          Criteria for diagnosis of Behcet's disease

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            Behçet's syndrome: disease manifestations, management, and advances in treatment.

            The acne lesions characteristic of Behçet's syndrome are not sterile and are commonly observed in combination with arthritis. The two main nodular skin lesions--superficial thrombophlebitis and erythema nodosum--are equally frequent, and rather difficult to distinguish. Superficial thrombophlebitis is usually observed in combination with thrombosis in large veins, and thrombosis of the large veins usually clusters with dural sinus thrombi, which make up approximately 20% of all central nervous system (CNS) lesions of Behçet's syndrome. The remaining CNS lesions are parenchymal, mainly located in the brainstem, and associated with a graver prognosis than dural sinus thrombi. The presence of clinical clusters indicates that there are at least two pathogenetic pathways in Behçet's syndrome: a reactive arthritis pathway and a thrombophilia pathway. Research into the pathogenesis of Behçet's syndrome has shown that the most consistent genetic marker of Behçet's syndrome is HLA-B51; however, the genetic association of this true-to-form 'complex' disorder with HLA-B51 is only 20%, and a whole-genome study showed associations with 16 different loci. The severity of Behçet's syndrome and the mortality associated with it tend to decrease with time, and there is no associated increase in incidence of atherosclerosis. Although treatment of skin-mucosa manifestations, eye disease and pulmonary artery aneurysms has improved significantly in the past decades, the treatment of CNS lesions and thrombophilia are still problematic.
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              Epidemiology and clinical characteristics of Behçet's disease in the US: a population-based study.

              Behçet's disease (BD) is a rare, multisystem inflammatory disorder of unknown cause. Although well-documented in Eastern populations, epidemiologic data is scarce in North American countries. Here we describe the incidence and prevalence of BD in Olmsted County, Minnesota over 45 years. We identified an incidence cohort of subjects age >/=18 years who had a clinical diagnosis of and/or fulfilled the International Study Group (ISG) criteria for BD from January 1, 1960 to January 1, 2005. Age- and sex-specific incidence and prevalence were estimated and age- and sex-adjusted to the 2000 US total population. The study population was comprised of 13 subjects with BD; 11 fulfilled ISG criteria between 1960 and 2005. Mean +/- SD age was 31 +/- 9 years, and 69% were white. Point prevalence in 2000 was 5.2 per 100,000 population (95% confidence interval [95% CI] 0.64-9.84). The overall annual age- and sex-adjusted incidence of BD was 0.38 per 100,000 population (95% CI 0.17-0.59), with a higher incidence in women (0.51 per 100,000; 95% CI 0.17-0.84) than in men (0.26 per 100,000; 95% CI 0.004-0.52). Dermatologic lesions included oral ulcers (100%), genital ulcers (62%), erythema nodosum (46%), and papulopustular lesions (54%). Ocular lesions, vascular complications, and central nervous system involvement were present in 8, 3, and 3 subjects, respectively. Our study shows an overall incidence of 0.38 per 100,000 population, which is comparable with other Western populations. The prevalence of 5.2 per 100,000 population is similar to estimates reported in Western countries, but lower than that in countries along the Silk Road.
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                Author and article information

                Contributors
                Ahmedm.elzanaty@gmail.com
                Journal
                Thromb J
                Thromb J
                Thrombosis Journal
                BioMed Central (London )
                1477-9560
                6 July 2020
                6 July 2020
                2020
                : 18
                : 12
                Affiliations
                [1 ]GRID grid.267337.4, ISNI 0000 0001 2184 944X, Internal Medicine Departement, , University of Toledo, ; 3000 Arlington Avenue, Toledo, OH 43614 USA
                [2 ]GRID grid.267337.4, ISNI 0000 0001 2184 944X, Cardiology Departement, , University of Toledo, ; Toledo, Ohio USA
                [3 ]GRID grid.479691.4, Cardiology Departement, , Tanta University Hospital, ; Tanta, Egypt
                Article
                225
                10.1186/s12959-020-00225-y
                7336494
                fcaeeb5b-c925-42ef-94d5-c5fbb943b194
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 25 April 2020
                : 10 June 2020
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2020

                Cardiovascular Medicine
                svc thrombosis,dilated cardiomyopathy,behcet’s disease
                Cardiovascular Medicine
                svc thrombosis, dilated cardiomyopathy, behcet’s disease

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