Behcet's Syndrome: Clinical Presentations Affecting Prognosis and Survival

Abstract

Behcet's syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. It has a high prevalence in the Middle-Eastern and Mediterranean countries. Recurrent skin-mu cosa lesions and sight-threatening panuveitis are the disease hallmarks. BS may also involve joints, vessels of all types and size, the central nervous system (CNS), and the gastrointestinal system. The disease course is characterized by exacerbations and remissions. The disease runs a more severe course among young males, and the severity diminishes with age. Males are more severely affected. Ocular, vascular, and CNS involvement are the main causes of morbidity. This may result in irreversible damage such as loss of useful vision, neurological disability, and potentially fatal bleeding from pulmonary artery aneurysm or Budd Chiari syndrome. Mortality rate is also increased in BS, especially in young males. Large vessel and parenchymal CNS disease are the main causes of death. However, an early and aggressive approach can lead to better treatment for the ocular and vascular diseases.