BIOLOGICAL THERAPY IN REFRACTORY NEUROBEHÇET’S DISEASE. MULTICENTER STUDY OF 42 PATIENTS

Background: Neuro-Behçet’s disease (NBD) is a severe complication of Behcet’s disease (BD). Despite well-established therapies with glucocorticoids and conventional immunosuppressants (cIS), a significant proportion of patients are refractory. Objectives: To assess efficacy and safety of biologic therapy (BT) in NBD refractory to glucocorticoids and at least one cIS. Methods: Open-label multicenter study of refractory NBD from 23 different referral Spanish Hospitals. Main outcome was neurological response. Secondarily, analytical efficacy was measured by Erythrocyte Sedimentation Rate (ESR), C-Reactive Protein (CRP) and Hemoglobin (Hb) at baseline, 6 months, 1 year and 2 years. Results: We studied 42 patients (21 women/ 21 men; mean age 40.4±10.8 years). HLA B51 was positive in 15 out of 37 (40.5%) patients tested. Non-neurological manifestations were oral ulcers (n=41, 97.6%), genital ulcers (n=31, 73.8%), skin lesions (n=28, 66.7%), arthralgia (n=27, 64.3%), uveitis (n=21, 50.0%), arthritis (n=9, 21.4%), venous thrombosis (n=9, 21.4%) and arterial thrombosis (n=4, 9.5%). The underlying neurologic manifestation were parenchymal (n=34, 81.0 %) and non-parenchymal (n=17, 40.5%) involvement ( Table 1 ). The first BT used was infliximab (n=20), adalimumab (n=13), golimumab (n=3), tocilizumab (n=3) and etanercept (n=2). After 58.2±51.4 months since initiation of BT, neurological response was complete (n=27; 64.3%), or partial (n=11, 26.1%) ( Figure 1 ). Only 4 (9.5%) patients did not respond. After 6 months of BT, ESR improved from.31.5±25.6 to 15.3±11.9 mm/h (p=0.005), CRP from 1.4 [0.2-12.8] to 0.3[0.1-3] mg/dL (p= 0.002) and Hb from 13.1±1.6 to 13.8±1.3 g/dL (p=0.005). Figure 1. Neurological clinical response to biological therapy. Primary failure was observed in 16 (38.1%) patients due to inefficacy (n=11, 68.8%) or adverse effects (n=5, 31.3%). Similarly, causes of secondary failure (n=6, 14.3%) were inefficacy (n=5, 83.3%) and adverse effects (n=1, 16.7%). No serious adverse effects were observed. Conclusion: BT, especially monoclonal anti-TNF drugs, seems to be effective and safe in refractory NBD. Table 1. Neurologic manifestation of 42 patients with refractory neurobehçet's disease treated with biologic therapy. Parenchymal subtype, n (% ) 34 (81.0 ) -Hemiparesis 8 (19.1) -Polineuropathy 8 (19.1) -Encephalopathy 6 (14.3) -Cognitive impairments 4 (9.5) -Optic neuropathy 4 (9.5) -Ophtalmoparesis 4 (9.5) -Other cranial nerve involvement 3 (7.1) -Hemihypoesthesia 3 (7.1) -Cerebellar dysphasia 1 (2.4) -Cerebellar involvement 1 (2.4) -Non-steroidal psicosis 1 (2.4) Non-parenchymal subtype, n (% ) 17 (40.5 ) -Aseptic meningitis 12(28.6) -Thrombosis 4 (9.5) -Intracranial hypertension 1 (2.4) Disclosure of Interests: Alba Herrero-Morant: None declared, José Luis Martín-Varillas Grant/research support from: AbbVie, Pfizer, Lilly, Janssen, and Celgene, Santos Castañeda: None declared, Iñigo González-Mazón: None declared, Olga Maiz: None declared, Ana Blanco Speakers bureau: AbbVie, Julio Sánchez: None declared, Norberto Ortego: None declared, Enrique Raya Speakers bureau: MSD, Grant/research support from: AbbVie, Alejandro Olive: None declared, Anahy Brandy-Garcia: None declared, Águeda Prior-Español: None declared, Clara Moriano: None declared, Elvira Diez Alvarez: None declared, Rafael Melero: None declared, Jenaro Graña: None declared, Álvaro Seijas-López: None declared, ANA URRUTICOECHEA-ARANA: None declared, Angel Ramos Calvo: None declared, Concepción Delgado Beltrán: None declared, Marta Loredo Martínez: None declared, Eva Salgado-Pérez: None declared, Francisca Sivera: None declared, Ignacio Torre-Salaberri: None declared, J. Narváez Speakers bureau: Bristol-Myers Squibb, José Luis Andréu Sánchez: None declared, Olga Martínez González: None declared, Ricardo Gómez de la Torre: None declared, Sabela Fernández: None declared, Susana Romero-Yuste: None declared, Gerard Espinosa: None declared, Miguel Á. González-Gay Speakers bureau: AbbVie, Pfizer, Roche, Sanofi, Lilly, Celgene and MSD, Grant/research support from: AbbVie, MSD, Jansen and Roche, Ricardo Blanco Speakers bureau: AbbVie, Pfizer, Roche, Bristol-Myers, Janssen, Sanofi, Lilly and MSD, Grant/research support from: AbbVie, MSD, and Roche Citation: Ann Rheum Dis, volume 80, supplement 1, year 2021, page 967Session: Other orphan diseases (POSTERS only)