Efficacy of TNF {alpha} blockade in cyclophosphamide resistant neuro-Behçet disease

Behçet disease is a chronic relapsing inflammatory condition, predominantly affecting young adults, characterised by recurrent bipolar aphtae and systemic manifestations for which tumour necrosis factor (TNF) alpha blockade has recently emerged as an effective treatment. We report the case of a patient presenting with mucocutaneous and ocular manifestations who in the course of his disease developed CNS parenchymal involvement. While being treated with pulsed cyclophosphamide and corticosteroids, he suffered a relapse of his CNS involvement that was efficaciously controlled by infliximab. No disease activity was observed during a full year of TNF blockade, associated with azathioprine, colchicine, and corticosteroids. However, 7 months after the last administration of infliximab and still under immunosuppressant agents, CNS lesions recurred. Infliximab was successfully reintroduced and since continued with no side effects. The sequence of events observed in this patient suggests that TNF blockade is efficacious in suppressing neuro-Behçet disease and once introduced should be maintained for a prolonged period of time.