Behçet's disease. Report of 41 cases and a review of the literature

Abstract

Several important conclusions may be derived on the basis of our experience with Behcet's disease (B.D.) and a review of the literature, namely: 1. B.D. is a systemic disease characterized by exacerbations and remissions of unpredictable duration, which affects mainly males between the ages of 20 to 30 years. Although its incidence is higher in the eastern hemisphere cases presented in all parts of the world. 2. The common histopathological lesion of all clinical manifestations appears to be a vasculitis. The etiology of B.D. is unknown. A viral etiology has been proposed but not confirmed. The presence of autoantibodies and lymphocyte sensitization to mucosal antigen has been observed but the precise significance of these findings in relation to the pathogenesis of the disease is presently undetermined. 3. The clinical manigestations present in most of the patients and considered diagnostic for B.D. are oral and genital ulcers, uveitis and skin lesions (especially erythema nodosum-like lesions or non-specific skin reactivity to needle pricks). The presence of three of the above manifestations are obligatory for the diagnosis. The other common clinical manifestations occurring in B.D. are: arthritis (44%); thrombophlebitis (24%), and various neurological syndromes (18%). Less frequent complications of the disease include: arterial thrombosis and aneurysm of both the systemic and pulmonary circulation, colitis, epididymitis and orchitis. 4. The serious chronic sequelae of this disease consist of blindness in up to 33% of patients with uveitis, vena caval obstruction and paralysis of limbs. Mortality, usually caused by meningoencephalitis, is rare. 5. The patients may present to different medical specialities, e.g., dermatology, gynecology, ophthalmology, neurology and internal medicine. To facilitate the correct diagnosis the essential criteria should be actively sought as they are not always the cause for medical consultation. 6. The diagnosis of B.D. is based only on clinical grounds as there are no pathognomonic laboratory or histopathological features. 7. Evaluation of treatment in B.D. is difficult, because of the naturally unpredictable course of the disease. Many drugs have been tried in the treatment of B.D. including antibiotics, anti-inflammatory drugs and corticosteroids, all with equivocal effects. Recently several additional treatment schedules have been suggested. Immunosuppressive drugs have been shown to have some beneficial effect, especially on uveitis. Fresh blood or plasma transfusions may prolong remissions. 8. The finding of decreased plasma fibrinolytic activity in patients with active B.D. represents the first observation of a possible disturbance in the blood coagulation mechanism and suggests that the use of fibrinolytic agents may be rewarding.