1558: Prevalence, Phenotypical Clinical Clusters and Treatment of Neurobehçet's Disease: Study in Northern Spain

Alba Herrero-Morant¹, Carmen Alvarez Reguera², Lara Sánchez-Bilbao², David Martínez-López², guillermo Suárez-Amorin², Raúl fernández-ramón², José Luis Martín-Varillas³, Cristina Mata⁴, Miguel Ángel González-Gay⁵ and Ricardo Blanco⁶, ¹Hospital Universitario Marqués de Valdecilla, Ontinyent, Spain, ²Hospital Universitario Marqués de Valdecilla, Santander, Spain, ³Hospital de Laredo, Laredo, Cantabria, Spain, ⁴Hospital Laredo, Santander, Spain, ⁵Department of Medicine and Psychiatry, Universidad de Cantabria; Rheumatology Division, Hospital Universitario Marqués de Valdecilla; Research group on genetic epidemiology and atherosclerosis in systemic diseases and in metabolic diseases of the musculoskeletal system, IDIVAL, Santander, Spain. Cardiovascular Pathophysiology and Genomics Research Unit, School of Physiology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa, ⁶Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

Background/Purpose: Behçet's disease (BD) may present with different clinical phenotypes. Ocular and Neurobehçet's Disease (NBD) are severe complications. Data on NBD epidemiology, clinical phenotype and therapy are scarce and controversial. In a wide unselected single-center series of BD our aims were to assess a) NBD prevalence, b) associations with other clinical clusters and c) treatment.

Methods: Cross-sectional study of all 120 patients diagnosed with BD in Northern Spain, between January 1, 1999 to December 31, 2019. Finally, 96 patients were included in this study according to 2014 International Criteria for Behçet Disease (ICBD). NBD was diagnosed according to the International Consensus Recommendation (ICR) criteria.

Results: NBD was diagnosed in 23 of 96 (24%) patients (15 women/8 men) (mean age: 44±13.9 years). NBD was classified as parenchymatous (n=10, 43.5%), non-parenchymatous (n=10, 43.5%) and mixed (n=3, 13%). HLAB51 was positive in 5 out of 13 (38.4%) patients tested. The main cluster of clinical associations were oral aphthae (n=20, 87%); ocular (n=14, 60.9%), cutaneous (n=10, 43.5%), articular (n=9, 39.1%), vascular (n=4, 17.4%) and intestinal (n=1, 8.7%) involvement (FIGURE). Treatments were oral corticosteroids (n=16; 69.6%; mean maximum dose 42±12.5 mg/ day, conventional immunosuppressants (n=13, 56.5%) and Biological Therapy (BT) (n= 7; 30.4%). BT was used in patients who were refractory to conventional immunosuppressants. Monoclonal anti-TNFα were used as the first option in all patients who received BT. In 3 out of 7 (42.7%) patients BT was switched due to inefficacy. TABLE shows the main NBD clinical subtypes and treatment. Complete remission was achieved in 18 of 23 cases (78.2%), partial response in 2 out of 23 cases (8.7%). No severe adverse effects were observed.

Conclusion: NBD was observed in 24% of patients with BD. The most frequent clinical clusters of NBD were oral aphthae and ocular involvement. All patients treated with either conventional immunosuppressant or BT achieved clinical remission.
TABLE: Main clinical features and treatment of 23 patients with Neurobehçet’s disease

FIGURE: Clusters of Neurobehçet’s clinical associations
Disclosures: A. Herrero-Morant, None; C. Alvarez Reguera, None; L. Sánchez-Bilbao, Eli Lilly; D. Martínez-López, None; g. Suárez-Amorin, None; R. fernández-ramón, None; J. Martín-Varillas, AbbVie/Abbott, Pfizer, Janssen, UCB, Celgene; C. Mata, None; M. González-Gay, AbbVie/Abbott, Merck/MSD, Janssen, Roche, AbbVie/Abbott, Roche, Sanofi, Eli Lilly, Celgene, Sobi, Merck/MSD; R. Blanco, Eli Lilly, Pfizer, Roche, Janssen, MSD, AbbVie, Amgen, AstraZeneca, Bristol Myers Squibb, Galapagos, Novartis, Sanofi.