[The eye and Behçet's disease]

Abstract

Behçet's disease (BD) is a systemic inflammatory disorder for which the underlying histopathology is an occlusive vasculitis. This disease is more common in the Mediterranean region, the middle east and the far east. It is characterized by oral and genital ulcerations, ocular inflammatory involvement, skin lesions, vascular involvement and numerous systemic manifestations that may affect mortality. The pathogenesis of the disease still remains poorly characterized, although infectious, genetic (HLA-B51 antigen polymorphism), and environmental factors have been implicated. Uveitis is by far the most common ocular manifestation of BD. It is an important diagnostic criterion and may be associated with severe visual loss. Management of both the patient's ocular and extra-ocular involvement requires close collaboration between the ophthalmologist and internist. Treatment of Behçet's uveitis has been better defined by the EULAR recommendations. It relies on azathioprine and systemic corticosteroids, the timely and more controlled institution of which has largely contributed to the improved visual prognosis. If the patient has severe eye disease, it is recommended that either cyclosporine A or infliximab be used in combination with azathioprine and corticosteroids; alternatively IFNα with or without corticosteroids may be used instead. The introduction of biological agents into the therapeutic armamentarium represents a significant advance in the treatment of severe refractory forms of the disease, compared to conventional treatment. A better understanding of the process of auto-immunity and the role of cytokines responsible for tissue damage in Behçet's disease and uveitides in general will allow new, more specific and effective therapeutic approaches to emerge in the near future.