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- alpha 2a (rhIFN-alpha2a) in two male adolescents suffering from severe treatment-resistant Behçet'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 Behçet'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-alpha2a was administered initially at 3 million IU/day for 4 weeks followed by 3 x 3 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.


CONCLUSIONS
RhIFN-alpha2a 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-alpha2a at an earlier time point needs to be considered.
Kuemmerle-Deschner
J B
JB
Division of Paediatric Rheumatology, University Children's Hospital Tuebingen, Germany. kuemmerle.deschner@uni-tuebingen.de
Tzaribachev
N
N
Deuter
C
C
Zierhut
M
M
Batra
M
M
Koetter
I
I
D000293:Adolescent; D001528:Behcet Syndrome / Q000188:drug therapy* / Q000473:pathology; D001921:Brain / Q000473:pathology; D002493:Central Nervous System Diseases / Q000188:drug therapy* / Q000473:pathology; D004351:Drug Resistance; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D000077190:Interferon alpha-2; D016898:Interferon-alpha / Q000627:therapeutic use*; D008279:Magnetic Resonance Imaging; D008297:Male; D011994:Recombinant Proteins; D016896:Treatment Outcome
D007166:Immunosuppressive Agents; D000077190:Interferon alpha-2; D016898:Interferon-alpha; D011994:Recombinant Proteins