1556: Distinct Clinical and Therapeutic Features Between Parenchymal and Non-parenchymal Manifestations in Neurobehçet's Disease

Alba Herrero-Morant¹, José Luis Martín-Varillas², Santos Castañeda³, Olga Maiz⁴, Julio Sánchez-Martín⁵, Norberto Ortego⁶, Enrique Raya⁷, Águeda Prior-Español⁸, Clara Moriano⁹, Rafael Melero¹⁰, Genaro Graña-Gil¹¹, Ana Urruticoechea¹², Angel Ramos¹³, Marta Loredo-Martínez¹⁴, Eva Salgado-Pérez¹⁵, Francisca Sivera¹⁶, Ignacio Torre¹⁷, F. Javier Narváez¹⁸, Jose Luis Andreu¹⁹, Olga Martinez²⁰, Ricardo Gómez-de la Torre²¹, Sabela Fernandez-Aguado²², Susana Romero Yuste²³, iñigo Gonzalez-Mazon⁵, Carmen Alvarez Reguera⁵, David Martínez-López⁵, José Luis Hernández⁵, Miguel Ángel González-Gay²⁴ and Ricardo Blanco²⁵, ¹Hospital Universitario Marqués de Valdecilla, Ontinyent, Spain, ²Hospital de Laredo, Laredo, Cantabria, Spain, ³Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain, ⁴Hospital Universitario de Donostia, San Sebastián, Spain, ⁵Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁶Medicine Department, Universidad de Granada, Granada, Spain, ⁷Hospital San Cecilio, Granada, Spain, ⁸Hospital Universitario Germans Trias i Pujol, Barcelona, Spain, ⁹Complejo Asistencial Universitario de León, León, Spain, ¹⁰Complexo Hospitalario Universitario de Vigo, Vigo, Spain, ¹¹Hospital Universitario de A Coruña, A Coruña, Spain, ¹²Hospital Can Misses, Ibiza, Spain, ¹³Complejo Hospitalario de Soria, Soria, Spain, ¹⁴Hospital Clínico Lozano Blesa, Zaragoza, Spain, ¹⁵Complejo Hospitalario Universitario de Ourense, Santiago de Composte, Spain, ¹⁶Hospital Universitario de Elda, San Vicente del Raspeig, Spain, ¹⁷Hospital de Basurto, Basurto, Spain, ¹⁸Rheumatology Department, Hospital Universitario de Bellvitge, Barcelona, Spain, ¹⁹Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain, ²⁰Hospital Clínico Universitario de Salamanca, Zamora, Spain, ²¹Hospital Universitario Central de Asturias, Oviedo, Spain, ²²Hospital Universitario de Cabueñes, Gijon, Spain, ²³Complexo Hospitalario Universitario, Pontevedra, Spain, ²⁴Department 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, ²⁵Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain

Background/Purpose: Ocular and Neurobehçet's Disease (NBD) are the most severe manifestations of Behcet's disease. NBD can be classified as a) primary neural parenchymal lesions, also known as parenchymal NBD (p-NBD) or b) secondary to vascular involvement or non-parenchymal NBD (np-NBD). Response to biologic therapy (BT) in these two refractory subtypes of NBD is unknown. The purpose of the study was to assess efficacy and safety of BT in refractory subtypes of NBD.

Methods: Open-label multicenter study of refractory NBD from 21 different referral National Hospitals. NBD diagnosis was based on the International Consensus Recommendation criteria (4). Efficacy was determined by complete or partial response and no-response. Complete, partial or no response was defined according to the resolution of the neurological syndrome (signs and/or symptoms) after the BT onset.

Results: We studied 41 patients (21 women/20 men; mean age: 40.6±10.8 years). NBD was classified as p-NBD (n= 33, 80.5%) and np-NBD (n=17, 41.5%). There were no significant differences in baseline general features and in neurological clinical response in both subgroups (TABLE and FIGURE). The first BT used in p-NBD were Infliximab (IFX) (n=15), Adalimumab (ADA) (n=11), Golimumab (GLM) (n=3), Tocilizumab (TCZ) (n=2) and Etanercept (ETN) (n=2) and in np-NBD were IFX (n=9), ADA (n=6), TCZ (n=1) and ETN (n=1).

After an overall mean follow-up of 57.5±50.9 months BT was switched in 22 patients due to inefficacy (n=16) or Adverse Effects (AE) (n=6) and in 4 cases was definitively discontinued because of complete prolonged remission (n=3) or AE (n=1). AE were observed in 7 (17.1%) patients. Severe AE were observed in 2 cases, one due to demyelinating disease and the other due to pulmonary tuberculosis, both in patients undergoing IFX therapy. The other 6 AE were infusion reaction to IFX (n=1), IFX-induced psoriasis (n=1), IFX-induced acneiform eruption (n=1), infusion reaction to TCZ (n=1), intolerance to IFX and recurrent mild infections (n=1) and erosive lichen planus and bullous impetigo (n=1).

Conclusion: In our series, BT seems equally effective and safe in both refractory p-NBD and np-NBD.
TABLE: Main features between p-NBD and np-NBD

FIGURE: Response to biological therapy according to NBD subtypes
Disclosures: A. Herrero-Morant, None; J. Martín-Varillas, AbbVie/Abbott, Pfizer, Janssen, UCB, Celgene; S. Castañeda, Roche; O. Maiz, None; J. Sánchez-Martín, None; N. Ortego, None; E. Raya, None; Á. Prior-Español, None; C. Moriano, None; R. Melero, None; G. Graña-Gil, None; A. Urruticoechea, None; A. Ramos, None; M. Loredo-Martínez, None; E. Salgado-Pérez, None; F. Sivera, None; I. Torre, None; F. Narváez, None; J. Andreu, None; O. Martinez, None; R. Gómez-de la Torre, None; S. Fernandez-Aguado, None; S. Romero Yuste, Pfizer, Lilly, AbbVie, Biogen, Sanofi; i. Gonzalez-Mazon, None; C. Alvarez Reguera, None; D. Martínez-López, None; J. Hernández, 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.