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[Behcet's disease: a rarely recognized orphan disorder].

作者信息

Kiss Emese, Dohán Judit, Németh János, Poór Gyula

机构信息

Országos Reumatológiai és Fizioterápiás Intézet Budapest Frankel Leó út 38-40. 1023 Semmelweis Egyetem, Általános Orvostudományi Kar III.

出版信息

Orv Hetil. 2013 Jan 20;154(3):93-101. doi: 10.1556/OH.2013.29528.

Abstract

Behcet's disease is a multisystem autoimmune disease with variable clinial manifestations. The diagnosis may pose a difficult challenge for the clinician, who has to be familiar with the wide spectrum and combination of the symptoms of Behcet's disease. It is considered a rare disease in Hungary, and there are only few reports on Behcet's disease in the Hungarian literature. However, the past history of Hungary, the worldwide growing incidence of the disease, and the authors' experience raise the possibility that the occurrence of the disease is higher than previously thought. In this review the authors present and discuss literature data on the pathogenesis and pathomechanism, as well as their own experience concerning the symptomatology of Behcet's disease in order to promote diagnosis and offer adequate therapy for the patients. The authors presume that the importance of the disease is underestimated in Hungary due to a considerable number of unrecognized cases and they propose to establish a national registry for Behcets disease.

摘要

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