Rituximab as an effective therapeutic option in refractory Neuro-Behçet syndrome

Behçet's disease (BD) is an inflammatory disease of unknown etiology with periods of relapses and remissions. Neuro-Behçet syndrome (NBS) is one of the main causes of long-term morbidity and mortality, making its prompt recognition and early treatment fundamental to achieving a better outcome. Currently there are no treatment guidelines either for BS or NB, making the management of these patients particularly difficult.

We present the case report of a patient with pseudo-tumoral lesion and myelitis refractory to steroids and cyclophosphamide who successfully showed remission after treatment with an anti-CD20 therapy.

This is the first report of concomitant pseudo-tumoral lesion and myelitis secondary to BS. We found rituximab treatment to be a safe and effective therapeutic option for NB supported by the radiological and clinical improvement achieved in our patient.