Behçet's
disease (BD) is a chronic, relapsing, and
debilitating systemic vasculitis of unknown
aetiology with the clinical features of
mucocutaneous lesions, ocular, vascular,
articular, neurologic, gastrointestinal,
urogenital, and pulmonary involvement. The
disease is much more frequent along the ancient
“Silk Route” extending from Eastern
Asia to the Mediterranean basin, compared with
Western countries. The disease usually starts
around the third or fourth decade of life. Male
sex and a younger age of onset are associated
with more severe disease. Although the
treatment has become much more effective in
recent years, BD is still associated with severe
morbidity and considerable mortality. The main
aim of the treatment should be the prevention of
irreversible organ damage. Therefore, close
monitoring, early, and appropriate treatment is
mandatory to reduce morbidity and mortality. The
treatment is mainly based on the suppression of
inflammatory attacks of the disease using
immunomodulatory and immunosuppressive agents.
In this paper, current state of knowledge
regarding the therapeutic approaches is
outlined. To provide a rational framework for
selecting the appropriate therapy along the
various treatment choices, a stepwise,
symptom-based, evidence-based algorithmic
approach was developed.