DefinePK

Abstract

Behçet's disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, gastrointestinal, urogenital, pulmonary, and neurologic involvement. Mucocutaneous lesions figure prominently in the presentation and diagnosis, and may be considered the hallmarks of BD. Therefore, their recognition may permit earlier diagnosis and treatment. 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. Traditional and current treatments with topical, paraocular and systemic corticosteroids, colchicine, dapsone, cyclosporine, azathioprine, methotrexate, cyclophosphamide and chlorambucil are summarized and recent insights into the pharmacology and effects of thalidomide, tacrolimus (FK-506), interferon-α, anti-TNF-α blocking monoclonal autoantibody (infliximab) and soluble TNF receptor (etanercept) are reviewed. We reviewed the current state of knowledge regarding the therapeutic approaches for BD and designed a stepwise, symptom-based, algorithmic approach, mainly based on controlled studies and our clinical experience in this field to provide a rational framework for selecting the appropriate therapy along the various treatment choices. Key clinical investigations with the status of ongoing clinical trials aimed at addressing the drug’s efficacy, surgical care, and studies that have raised the possibility of new therapeutic uses are also presented. The challenges posed by the drug’s teratogenicity and adverse effects are also considered, if present.