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Abstract Number: 1693

Biological Therapy in Neurobehçet: Multicenter Study of 31 Patients

Iñigo Gonzalez-Mazon1, Lara Sanchez-Bilbao 2, Jose Luis Martín-Varillas 2, Belén Atienza-Mateo 2, Vanesa Calvo Río 2, Santos Castañeda 3, Esther Vicente-Rabaneda 4, Olga Maiz 5, ana blanco 5, Clara Moriano 6, Elvira Díez 6, JL Andreu 7, Concepción Delgado-Beltrán 8, Marta Loredo-Martinez 8, Javier Narváez 9, Angel Ramos-Calvo 10, Francisca Sivera 11, E. Raya 12, Norberto Ortego Centeno 12, Jose luis callejas rubio 12, Anahy Brandy-Garcia 13, Alejandro Olivé-Marqués 14, Sabela Fernández 15, Ricardo Gómez De La Torre 16, Iñaki Torre-Salaberri 17, Julio Sanchez 18, A. Urruticoechea-Arana 19, Eva salgado-Pérez 20, Rafael Melero 21, Olga Martinez 22, Susana Romero-Yuste 23, Miguel A Gonzalez-Gay 2 and Ricardo Blanco 2, 1Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain, 2Rheumatology, Hospital Universitario Marques de Valdecilla, Santander, Spain, 3Rheumatology, Hospital Universitario La Princesa, Madrid, Madrid, Spain, 4Rheumatology Department, Hospital Universitario La Princesa, Madrid, Spain, 5Rheumatology, Hospital Universitario Donostia, san sebastian, Pais Vasco, Spain, 6Complejo Asistencial Universitario de León, León, Castilla y Leon, Spain, 7HU Puerta del Hierro, Madrid, 8H. Clinico Lozano Blesa Zaragoza, Zaragoza, Aragon, Spain, 9Rheumatology Department, Hospital Universitari de Bellvitge, Barcelona, Catalonia, Spain, 10Rheumatology, Complejo Hospitalario de Soria, soria, Castilla y Leon, Spain, 11Hospital General Universitario de Elda, Elda, Spain, 12Hospital San Cecilio, Granada, Andalucia, Spain, 13Hospital Universitari Germans Trias i Pujol, Badalona, Spain, 14Hospital Universitari Germans Trias i Pujol, Badalona, Catalonia, Spain, 15Hospital Universitario San Agustín, Avilés, Asturias, Spain, 16Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain, Oviedo, Spain, 17BASURTO UNIVERSITY HOSPITAL, Bilbao, Spain, 18H. 12 de Octubre, Madrid, Spain, 19HU Can Misses, Ibiza, Spain, 20Complejo Hospitalario Universitario de Ourense, Ourense, Galicia, Spain, 21Complexo Hospitalario Universitario de Vigo, Vigo, Galicia, Spain, 22Hospital Clínico Universitario de Salamanca, Salamanca, Spain, 23Complejo Hospitalario Universitario Pontevedra, Pontevedra, Galicia, Spain

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Behcet's syndrome and vasculitis, Neurologic involvement

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Session Information

Date: Monday, November 11, 2019

Session Title: Vasculitis – Non-ANCA-Associated & Related Disorders Poster III: Behçet’s Disease & Other Vasculitides

Session Type: Poster Session (Monday)

Session Time: 9:00AM-11:00AM

Background/Purpose: Behçet’s disease (BD) is a variable vessel vasculitis and typically presents with mucocutaneous involvement. However, any organ can be affected, being the neurological affectation (neurobehçet, NB) one of the most serious manifestations.
Our aim was to assess the efficacy and safety of biological therapy as treatment of NB.

Methods: We set up a multicenter observational study of 31 patients with NB on treatment with biological therapy (BT). NB diagnosis was made by neuroimaging, CSF analysis and/or suggestive clinical signs of central and/or peripheral nervous system involvement, excluding infectious causes or more prevalent pathology. Results are expressed as mean±SD or as median and interquartile range (IQR) as appropriate.

Results: 31 patients (16♂ / 15♀) with an average age of 39.7 ± 10.5 years. HLA-B51 was positive in 51.6% of the patients. Table shows the non-neurological manifestations. Regarding the neurological manifestations, 23 patients (74.2%) had parenchymal involvement (hemiparesis (n=6), brainstem involvement (n=1), encephalopathy (n = 4), optic neuropathy (n=3), dysphasia (n=1), polyneuropathy (n=6), cognitive impairment (n=4), and non-steroidal psychosis (n=1), while the remaining 8 patients (25.8%) presented aseptic meningitis as a non-parenchymal affectation (Table).

Prior to BT, patients had received the following treatment: oral prednisone (n=29), methylprednisolone bolus (n=9), CsA (n=10), AZA (n=17), MTX (n=16) and mycophenolate (n=2).

After a median of 34 [10-72] months since the beginning of the neurological symptoms, the following BT was initiated: infliximab (IFX)(n=19), adalimumab (ADA)(n=7), tocilizumab (TCZ) (n=2), golimumab (GOL) (n=2) and Etanercept (ETN) (n=1). A first switch to ADA was necessary in 9 patients with IFX due to primary failure. In addition, 2 of them needed a second switch to TCZ, getting a partial response. The BT was discontinued in 5 patients, 2 of them for obtaining clinical remission and the remaining 3 for inefficacy.

After a median follow-up of 5.4±4.6 years, complete response was obtained in 15 patients, partial response in 11 and no response in the remaining 3. We observed an anaphylactic reaction and psoriasis induced by IFX, without other serious adverse events (Table).

Conclusion: BT, especially anti-TNF, seems effective and safe for treatment in NB.


Disclosure: I. Gonzalez-Mazon, None; L. Sanchez-Bilbao, None; J. Martín-Varillas, None; B. Atienza-Mateo, None; V. Calvo Río, None; S. Castañeda, None; E. Vicente-Rabaneda, None; O. Maiz, None; a. blanco, None; C. Moriano, None; E. Díez, None; J. Andreu, None; C. Delgado-Beltrán, None; M. Loredo-Martinez, None; J. Narváez, None; A. Ramos-Calvo, None; F. Sivera, None; E. Raya, None; N. Ortego Centeno, None; J. callejas rubio, None; A. Brandy-Garcia, None; A. Olivé-Marqués, None; S. Fernández, None; R. Gómez De La Torre, None; I. Torre-Salaberri, None; J. Sanchez, None; A. Urruticoechea-Arana, None; E. salgado-Pérez, None; R. Melero, None; O. Martinez, None; S. Romero-Yuste, None; M. Gonzalez-Gay, Abbvie, 2, 5, 8, Celgene, 5, 8, Janssen, 2, MSD, 2, 5, 8, Pfizer, 5, 8, Roche, 2, 5, 8, Sanofi, 5, 8, Sobi, 5, 8; R. Blanco, None.

To cite this abstract in AMA style:

Gonzalez-Mazon I, Sanchez-Bilbao L, Martín-Varillas J, Atienza-Mateo B, Calvo Río V, Castañeda S, Vicente-Rabaneda E, Maiz O, blanco a, Moriano C, Díez E, Andreu J, Delgado-Beltrán C, Loredo-Martinez M, Narváez J, Ramos-Calvo A, Sivera F, Raya E, Ortego Centeno N, callejas rubio J, Brandy-Garcia A, Olivé-Marqués A, Fernández S, Gómez De La Torre R, Torre-Salaberri I, Sanchez J, Urruticoechea-Arana A, salgado-Pérez E, Melero R, Martinez O, Romero-Yuste S, Gonzalez-Gay M, Blanco R. Biological Therapy in Neurobehçet: Multicenter Study of 31 Patients [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/biological-therapy-in-neurobehcet-multicenter-study-of-31-patients/. Accessed April 13, 2021.
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