Safety and efficacy of etanercept in children with juvenile-onset Behcets disease

Behcets disease (BD) is a chronic, relapsing, multi-system inflammatory disorder, clinically characterized by recurrent oral and genital ulcers, skin lesions, and uveitis. Other manifestations include arthritis, a positive pathergy test, thrombophlebitis, central nervous system disease and gastrointestinal ulcerations. The majority of affected individuals do not have life-threatening disease, although mortality can be associated with vascular-thrombotic and neurological manifestations. Currently, treatment of BD is symptomatic and empirical, and is tailored according to the severity of clinical features. In the past few years, isolated reports and case-series have been published on adult BD patients suggesting that inhibition of TNF-alpha is a promising therapeutic approach for severe ocular and various extra-ocular manifestations, including central nervous system involvement. In this study we present our promising experience with Etanercept therapy in juvenile-onset BD patients, characterized by refractory multiorgan involvement.