Interferon-alpha--a new therapeutic option in refractory juvenile Behçet's disease with CNS involvement

Objective. To report the successful treatment with recombinant human IFN- 2a (rhIFN-2a) in two male adolescents suffering from severe treatment-resistant Behcet's disease (BD) with central nervous system (CNS) involvement. Methods. The patients were 14- and 15-yrs old. Both met the International Study Group for Behcet's disease, O'Duffy and the Japanese criteria for the classification or diagnosis of BD. Signs of CNS involvement were impaired sensorimotor function of the left arm, hemiparesis of right arm and leg, dizziness and walking instability in Patient 1, weakness of both legs, impaired bladder-, bowel- and sexual function in Patient 2 and vasculitic lesions on cranial MRI in both patients. RhIFN-2a was administered initially at 3 million IU/day for 4 weeks followed by 33 million IU/week. Results. Complete remission was achieved in Patient 1 (reduction in BD activity score from 17 to 2). Patient 2 experienced remarkable improvement (reduction of BD activity score from 23 to 15). In both patients the MRI lesions improved. Patient 2 had mild flu-like symptoms as adverse effect. Conclusion. RhIFN-2a was effective and well tolerated in these juvenile patients with severe neurological BD. Regarding the serious consequences following ocular and CNS affection and adverse effects of steroid dependency, administration of rhIFN-2a at an earlier time point needs to be considered.