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Prognosis in Behçet’s Syndrome

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Behçet’s Syndrome

Abstract

Behçet’s syndrome (BS) may cause several serious morbidities and a fatal outcome. A follow-up study of 20 years found an overall mortality rate of 10% among an inception cohort and increased standardized mortality ratios among young males. Major causes of death were large vessel and parenchymal central nervous system involvement. Mortality rate tends to decrease with time. The same trend is observed in the severity of eye disease and the frequency of skin-mucosa lesions. However, parenchymal CNS disease and peripheral artery aneurysms may occur late during the follow-up. Also, male patients with BS are more prone to develop serious morbidities at long term even when they have no major organ involvement during the early years of their disease. Major morbidity results from eye, vascular, and neurological involvement. The outcome of eye disease is improving significantly after 1990 compared to the era before azathioprine and cyclosporine. However, still about 10% of patients may go blind in 5 years despite treatment. Similarly, prognosis is still grim for pulmonary artery aneurysms, which lead to death in about ¼ of the affected cases and parenchymal neurological involvement, which results in death or severe disability in approximately half of the affected cases. Here we review several aspects of morbidity and mortality due to BS.

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Authors and Affiliations

  1. Division of Rheumatology, Department of Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Emire Seyahi

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  1. Emire Seyahi
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  2. Hasan Yazıcı
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Correspondence to Emire Seyahi .

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Editors and Affiliations

  1. Hospital for Joint Diseases, Behçet's Syndrome Center, NYU Medical Center, East 20th Street 246, New York, 10003, New York, USA

    Yusuf Yazıcı

  2. , Department of Medicine, University of Istanbul, Istanbul, 34452, Turkey

    Hasan Yazıcı

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Seyahi, E., Yazıcı, H. (2010). Prognosis in Behçet’s Syndrome. In: Yazıcı, Y., Yazıcı, H. (eds) Behçet’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5641-5_17

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  • DOI: https://doi.org/10.1007/978-1-4419-5641-5_17

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