Biological agents in the treatment of severe and/or refractory neuro-Behçet's disease

Abstract

Objective To explore the use of biological agents in Neuro-Behcet's disease (NBD). Methods We retrospectively reviewed the clinical data of five NBD patients treated with biological agents who were in-patients at Peking Union Medical College Hospital between June 2009 and June 2016. The continuous variables were analyzed by t test. Results All five cases (4 male and 1 female) were severe and/or refractory patients with parenchymal involvement (pNBD). Their age at neurological onset was (31±12) years old. Four cases presented with multiple lesions. The brainstem, spinal cord, cerebral hemisphere and cere-bellum involvement were presented in 4,3,3 and 2 patients, respectively. The Rankin score at the onset of NBD was (4.0±0.7). The biological agents were administrated when corticosteroids and immunosuppressant were ineffective. Three cases received tumor necrosis factor (TNF)-α inhibitors therapy, among whom one patient had gastrointestinal ulceration. One patient with refractory retinal vasculitis received interferon-α therapy. One patient treated with tocilizumab [interleukin (IL)-6R inhibitor] had high level IL-6 in the cerebrospinal fluid. All patients achieved clinical improvements and the Rankin score significantly decreased to (2.2±0.8) when compared with the baseline (t=4.81, P<0.01) after the treatment with biological agents. The corticosteroid dose was tapered in all cases and the numbers of immunosuppressants were reduced in most cases, indicating a potential steroid and immunosup-pressant-sparing effect. No serious adverse events were observed during the follow-up. Conclusion Neurological involvement is a severe complication of Behcet's disease. We can take appropriate biological agents such as TNF-α inhibitors or interferon-α into consideration when patients have severe/refractory pNBD. Key words: Behcet syndrome; Biological agents; Neuro-Behcet's disease