Efficacy of anti-TNF alpha in severe and/or refractory Behçet's disease: Multicenter study of 124 patients.

OBJECTIVE: To report the efficacy and safety of anti-TNF agents in patients with severe and/or refractory manifestations of Behçet's disease (BD). METHODS: We performed a multicenter study of main characteristics and outcomes of anti-TNF alpha treatments [mainly infliximab (62%), and adalimumab (30%)] in 124 BD patients [48% of men; median age of 33.5 (28-40) years]. RESULTS: Overall response (i.e. complete and partial) rate was 90.4%. Clinical responses were observed in 96.3%, 88%, 70%, 77.8%, 92.3% and 66.7% of patients with severe and/or refractory ocular, mucocutaneous, joint, gastro-intestinal manifestations, central nervous system manifestations and cardiovascular manifestations, respectively. No significant difference was found with respect to the efficacy of anti-TNF used as monotherapy or in association with an immunosuppressive agent. The incidence of BD flares/patient/year was significantly lower during anti-TNF treatment (0.2 ± 0.5 vs 1.7 ± 2.4 before the use of anti-TNF, p < 0.0001). The prednisone dose was significantly reduced at 6 and 12 months (p < 0.0001). In multivariate analysis, retinal vasculitis was negatively associated with complete response to anti-TNF (OR = 0.33 [0.12-0.89]; p = 0.03). The efficacy and relapse free survival were similar regardless of the type of anti-TNF agent used. After a median follow-up of 21 [7-36] months, side effects were reported in 28% of patients, including infections (16.3%) and hypersensitivity reactions (4.1%). Serious adverse events were reported in 13% of cases. CONCLUSION: Anti-TNF alpha therapy is efficient in all severe and refractory BD manifestations. Efficacy appears to be similar regardless of the anti-TNF agent used (infliximab or adalimumab).

Familial aggregation in Behçet's disease: high frequency in siblings and parents of pediatric probands.

OBJECTIVE: To examine familial aggregation of Behçet's disease (BD) in pediatric compared with non-pediatric patients. METHODS: A retrospective study was conducted to analyze data collected from 572 patients with BD in whom the diagnosis was made with criteria defined by the International Study Group for BD. The age of attaining criteria (the age at which the patient met the study group criteria) was evaluated for each patient. Recurrence risks were calculated for the pediatric group from information provided by 45 families. RESULTS: Of the 505 patients from whom the age of attaining criteria could be ascertained, 106 showed definitive BD before the age of 16 years and were considered as pediatric patients with BD; the other 399 were classified as non-pediatric patients. Thirteen of the 106 pediatric patients (12.3%) and only 9 of the 399 non-pediatric patients (2.2%) had relatives affected by BD. This excess of familial cases in the pediatric group compared with the non-pediatric group was significant (P <.0001, chi2 analysis). Moreover, the mean age of attaining criteria in familial cases (17. 95 years [SD = 8.62]) was significantly lower than in sporadic cases (27.28 years [SD = 11]; P <.0001, Student t test). The recurrence risk among siblings and parents who met the International Study Group criteria was 0.1. CONCLUSION: Our data support the hypothesis of a genetic component in the pathogenesis of BD, and we propose the inclusion of familial history in the definition of pediatric BD.