Biologic therapy in refractory neurobehçet's disease: a multicentre study of 41 patients and literature review.

OBJECTIVES: To assess efficacy and safety of biologic therapy (BT) in neurobehçet's disease (NBD) refractory to glucocorticoids and at least one conventional immunosuppressive drug. METHODS: Open-label, national, multicentre study. NBD diagnosis was based on the International Consensus Recommendation criteria. Outcome variables were efficacy and safety. Main efficacy outcome was clinical remission. Other outcome variables analysed were glucocorticoid-sparing effect and improvement in laboratory parameters. RESULTS: We studied 41 patients [21 women; age 40.6 (10.8) years]. Neurological damage was parenchymal (n = 33, 80.5%) and non-parenchymal (n = 17, 41.5%). First BTs used were infliximab (n = 19), adalimumab (n = 14), golimumab (n = 3), tocilizumab (n = 3) and etanercept (n = 2). After 6 months of BT, neurological remission was complete (n = 23, 56.1%), partial (n = 15, 37.6%) and no response (n = 3, 7.3%). In addition, median (IQR) dose of oral prednisone decreased from 60 (30-60) mg/day at the initial visit to 5 (3.8-10) mg/day after 6 months (P < 0.001). It was also the case for mean erythrocyte sedimentation rate [31.5 (25.6)-15.3 (11.9) mm/1st h, P = 0.011] and median (IQR) C-reactive protein [1.4 (0.2-12.8) to 0.3 (0.1-3) mg/dl, P = 0.001]. After a mean follow-up of 57.5 months, partial or complete neurological remission persisted in 37 patients (90.2%). BT was switched in 22 cases (53.6%) due to inefficacy (n = 16) or adverse events (AEs) (n = 6) and discontinued due to complete prolonged remission (n = 3) or severe AE (n = 1). Serious AEs were observed in two patients under infliximab treatment. CONCLUSIONS: BT appears to be effective and relatively safe in refractory NBD.

Aspectos clínicos novedosos en la enfermedad de Behçet / New clinical aspects of Behçet’s disease

La etiopatogenia del síndrome o enfermedad de Behçet (EB) sigue siendo esquiva y sus manifestaciones clínicas, muy complejas, y genera una buena cantidad de artículos científicos. Por ejemplo, una búsqueda simple en PubMed muestra que en los últimos 5 años se han publicado 1.394 artículos, 159 de ellos revisiones. Además de los estudios básicos sobre etiopatogenia y mecanismos de la enfermedad, las diversas manifestaciones clínicas y sus tratamientos, también es fuente de debate la propia definición de la enfermedad, su clasificación y los criterios de clasificación y diagnóstico. En este artículo revisamos algunos aspectos en debate, así como las últimas alternativas terapéuticas y la situación de la EB en España (AU)

Behçet’s disease/syndrome (BD) continues being aloof in his etiopathogenesis and very complex in its clinical manifestations. BD generates a good amount of scientific articles. For example, a simple search in PubMed sample that in the last 5 years has been published 1394 articles, 159 of them revisions. In addition to the basic studies on etiopahtogenesis and disease mechanisms, the diverse clinical manifestations and their treatments, also the own definition of the disease, its classification and the criteria of classification and diagnosis are debate source. In this article we reviewed some aspects in discussin as well as the last therapeutic alternatives and the situation of the EB in Spain (AU)