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The Prognosis of Behçet Syndrome

  • Emire Seyahi
  • David Saadoun
Chapter

Abstract

Behçet’s syndrome (BS) may cause several serious morbidities and a fatal outcome. A 20-year follow-up study found an overall mortality rate of 5–10% 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 disease course. 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 with biological agents. However, still about 10% of patients may become blind despite treatment. Similarly, prognosis is still grim for pulmonary artery aneurysms, which lead to death in about one fourth 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.

Keywords

Behçet’s syndrome Prognosis Mortality Morbidity Eye disease Vascular involvement Neurological involvement Cancer 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Emire Seyahi
    • 1
  • David Saadoun
    • 2
  1. 1.Division of Rheumatology, Department of Internal MedicineIstanbul University-CerrahpasaIstanbulTurkey
  2. 2.Department of Internal Medicine and Clinical ImmunologyCentre national de référence des maladies Autoimmunes Systémiques rares, Centre national de référence des maladies Autoinflammatoires et de l’Amylose; Groupe Hospitalier Pitié-Salpêtrière, AP-HP; Sorbonne Universités, UPMC Univ Paris 06ParisFrance

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