Biologic therapy in refractory Neurobehçet's disease: a multicenter study of 41 patients and literature review

• Published in: Rheumatology (Oxford, England)
• Main Authors: Herrero-Morant, Alba, Martín-Varillas, José Luis, Castañeda, Santos, Maíz, Olga, Sánchez, Julio, Ortego, Norberto, Raya, Enrique, Prior-Español, Águeda, Moriano, Clara, Melero-González, Rafael B, Graña-Gil, Genaro, Urruticoechea-Arana, Ana, Ramos-Calvo, Ángel, Loredo-Martínez, Marta, Salgado-Pérez, Eva, Sivera, Francisca, Torre, Ignacio, Narváez, Javier, Andreu, José Luis, Martínez-González, Olga, Gómez-de la Torre, Ricardo, Fernández-Aguado, Sabela, Romero-Yuste, Susana, González-Mazón, Íñigo, Álvarez-Reguera, Carmen, Hernández, José Luis, González-Gay, Miguel Ángel, Blanco, Ricardo
• Format: Journal Article
• Language: English
• Published: England 15.02.2022
• Subjects: anakinra; tocilizumab; biologic therapy; canakinumab; ustekinumab; Neurobehçet; rituximab; anti-TNF
• ISSN: 1462-0332

To assess efficacy and safety of biologic therapy (BT) in Neurobehçet's disease (NBD) refractory to glucocorticoids and at least one conventional immunosuppressive drug. Open-label, national, multicenter 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 analyzed were glucocorticoid-sparing effect and improvement in laboratory parameters. 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%), no response (n = 3, 7.3%). In addition, mean 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 to 15.3±11.9 mm/1st hour, p = 0.005) and median [IQR] C-reactive protein (1.4 [0.2-12.8] to 0.3 [0.1-3] mg/dL, p = 0.002). 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 2 patients under infliximab treatment. BT appears to be effective and relatively safe in refractory NBD.